Sharon Worcester

DESTIN, FLA. — Pregnant women with antiphospholipid syndrome need anticoagulation throughout pregnancy and for at least 6 weeks post partum, Dr. Ann Parke said at the annual Rheumatology on the Beach.

Low-dose aspirin can also be added to anticoagulant therapy. The antimalarial drug hydroxychloroquine should be continued in those with systemic lupus erythematosus (SLE), said Dr. Parke, professor of medicine at the University of Connecticut, Farmington.

Discontinuing hydroxychloroquine will put the patient at risk of an SLE flare, putting the pregnancy and patient in jeopardy. SLE flares, as well as pregnancy, are among the known “second hit” phenomena that can trigger thrombosis in patients with antiphospholipid antibodies, Dr. Parke said. (See related story.)

Furthermore, data suggest that this drug—which reverses platelet activation induced by human IgG anticardiolipin, and which has been shown to reduce thrombosis size in antiphospholipid animal models—is safe in pregnancy, she added.

Other treatments used in pregnant patients with antiphospholipid syndrome (APS) include corticosteroids and intravenous immunoglobulin. IVIG is expensive, and there is no clear evidence of benefit, said Dr. Parke. Corticosteroids, which lost favor because of associated increases in maternal morbidity and risk of preterm birth, may regain ground in light of new evidence that complement activation plays a role in antiphospholipid antibody-associated placental injury. (See box.)

Pregnant patients with APS should be monitored closely for signs of placental insufficiency, HELLP (hemolytic anemia, elevated liver enzymes, low platelet count) syndrome, and toxemia, she said, noting more than 80% of women with APS experience at least one fetal death; in fact, the specificity of fetal death for the presence of antiphospholipid antibodies is 76%.

As for the treatment of pregnant patients with antiphospholipid antibodies who don't meet the criteria for antiphospholipid syndrome, a literature review published last year (JAMA 2006;295:1050–7) suggested asymptomatic patients and those who have experienced a single fetal loss before 10 weeks' gestation should receive no treatment, and that those with recurrent losses but no clinical thrombotic event should receive low-dose aspirin and prophylactic heparin throughout pregnancy and for a minimum of 6 weeks post partum. (Dr. Parke said she suspects this will be increased to 4 months because recent evidence suggests the risk of thrombosis is high throughout the first postpartum year.)

Like her recommendations for those with APS, the findings from the literature review supported the use of low-dose aspirin and therapeutic heparin throughout pregnancy, plus long-term warfarin post partum in those with recurrent losses and a clinical thrombotic event (who thus meet current Sapporo Criteria for APS).

Warfarin use, however, depends on the nature of the antibodies present. Lupus anticoagulant is more likely to be associated with clinical events than is anticardiolipin, for example. Titer, persistence, and isotype of the antibodies; the number of positive antibodies; and the presence of additional risk factors, such as SLE, also play into the decision to prescribe warfarin. A number of studies have shown moderate warfarin doses are as effective as and safer than high doses in such patients. It remains unclear whether it is necessary to use warfarin in patients with antibodies who have a clinical thrombotic event triggered by second hit phenomenon, she said.

Other issues not adequately addressed include therapy for patients with antibodies but no clinical event, optimal therapy for noncerebral artery thrombosis, managing patients who have recurrence despite adequate international normalized ratios, and therapy in women with antibodies and recurrent fetal losses.

Studies Probe APLA, Complement Links

Animal studies show preventing complement activation is essential in pregnancy, and deficiencies of certain complement components in the presence of antiphospholipid antibodies (APLA) prevent fetal death. Inhibitors of these components are protective in antibody-exposed mice. A study of human placentas suggests complement activation plays a role in APLA-associated placental injury, Dr. Parke said.

Using immunohistochemical methods to identify deposition of complement activation products in the placentas of 47 full-term viable pregnancies in women with APLA and 23 control placentas, investigators showed those with antibodies had more deposition of complement components C4d, C3b, and C5b-9 in the villous trophoblast cytoplasm and the extravillous trophoblast of the basal plate, and deposition of C4d in the trophoblastic cell and basement membrane (Am. J. Obstet. Gynecol. 2007;196:167).

Immunoreactivity was stronger for the C4d protein in all three areas in the APLA placentas and for the C3b protein in the villous trophoblast cytoplasm in the APLA placentas, versus controls. A link was seen between pathologic lesions and the deposition of C4d in the trophoblast cytoplasm and cellular and basement membranes.

In light of prior data showing trophoblastic cell membranes are targets for APLA, and the finding that placental lesions in women with these antibodies are tied to malperfusion, it seems “proinflammatory factors that stimulate complement activation may precede the changes that ultimately lead to ischemia, tissue injury, and fetal loss,” they wrote.

The deposition of complement activation products in patients with antibodies occurs because the protection provided by complement regulatory proteins is overwhelmed by antibodies. This deposition is likely due to increased activation of the complement system, rather than a depletion or inactivation of the complement regulatory proteins.

DESTIN, FLA. — Pregnant women with antiphospholipid syndrome need anticoagulation throughout pregnancy and for at least 6 weeks post partum, Dr. Ann Parke said at the annual Rheumatology on the Beach.

Low-dose aspirin can also be added to anticoagulant therapy. The antimalarial drug hydroxychloroquine should be continued in those with systemic lupus erythematosus (SLE), said Dr. Parke, professor of medicine at the University of Connecticut, Farmington.

Discontinuing hydroxychloroquine will put the patient at risk of an SLE flare, putting the pregnancy and patient in jeopardy. SLE flares, as well as pregnancy, are among the known “second hit” phenomena that can trigger thrombosis in patients with antiphospholipid antibodies, Dr. Parke said. (See related story.)

Furthermore, data suggest that this drug—which reverses platelet activation induced by human IgG anticardiolipin, and which has been shown to reduce thrombosis size in antiphospholipid animal models—is safe in pregnancy, she added.

Other treatments used in pregnant patients with antiphospholipid syndrome (APS) include corticosteroids and intravenous immunoglobulin. IVIG is expensive, and there is no clear evidence of benefit, said Dr. Parke. Corticosteroids, which lost favor because of associated increases in maternal morbidity and risk of preterm birth, may regain ground in light of new evidence that complement activation plays a role in antiphospholipid antibody-associated placental injury. (See box.)

Pregnant patients with APS should be monitored closely for signs of placental insufficiency, HELLP (hemolytic anemia, elevated liver enzymes, low platelet count) syndrome, and toxemia, she said, noting more than 80% of women with APS experience at least one fetal death; in fact, the specificity of fetal death for the presence of antiphospholipid antibodies is 76%.

As for the treatment of pregnant patients with antiphospholipid antibodies who don't meet the criteria for antiphospholipid syndrome, a literature review published last year (JAMA 2006;295:1050–7) suggested asymptomatic patients and those who have experienced a single fetal loss before 10 weeks' gestation should receive no treatment, and that those with recurrent losses but no clinical thrombotic event should receive low-dose aspirin and prophylactic heparin throughout pregnancy and for a minimum of 6 weeks post partum. (Dr. Parke said she suspects this will be increased to 4 months because recent evidence suggests the risk of thrombosis is high throughout the first postpartum year.)

Like her recommendations for those with APS, the findings from the literature review supported the use of low-dose aspirin and therapeutic heparin throughout pregnancy, plus long-term warfarin post partum in those with recurrent losses and a clinical thrombotic event (who thus meet current Sapporo Criteria for APS).

Warfarin use, however, depends on the nature of the antibodies present. Lupus anticoagulant is more likely to be associated with clinical events than is anticardiolipin, for example. Titer, persistence, and isotype of the antibodies; the number of positive antibodies; and the presence of additional risk factors, such as SLE, also play into the decision to prescribe warfarin. A number of studies have shown moderate warfarin doses are as effective as and safer than high doses in such patients. It remains unclear whether it is necessary to use warfarin in patients with antibodies who have a clinical thrombotic event triggered by second hit phenomenon, she said.

Other issues not adequately addressed include therapy for patients with antibodies but no clinical event, optimal therapy for noncerebral artery thrombosis, managing patients who have recurrence despite adequate international normalized ratios, and therapy in women with antibodies and recurrent fetal losses.

Studies Probe APLA, Complement Links

Animal studies show preventing complement activation is essential in pregnancy, and deficiencies of certain complement components in the presence of antiphospholipid antibodies (APLA) prevent fetal death. Inhibitors of these components are protective in antibody-exposed mice. A study of human placentas suggests complement activation plays a role in APLA-associated placental injury, Dr. Parke said.

Using immunohistochemical methods to identify deposition of complement activation products in the placentas of 47 full-term viable pregnancies in women with APLA and 23 control placentas, investigators showed those with antibodies had more deposition of complement components C4d, C3b, and C5b-9 in the villous trophoblast cytoplasm and the extravillous trophoblast of the basal plate, and deposition of C4d in the trophoblastic cell and basement membrane (Am. J. Obstet. Gynecol. 2007;196:167).

Immunoreactivity was stronger for the C4d protein in all three areas in the APLA placentas and for the C3b protein in the villous trophoblast cytoplasm in the APLA placentas, versus controls. A link was seen between pathologic lesions and the deposition of C4d in the trophoblast cytoplasm and cellular and basement membranes.

In light of prior data showing trophoblastic cell membranes are targets for APLA, and the finding that placental lesions in women with these antibodies are tied to malperfusion, it seems “proinflammatory factors that stimulate complement activation may precede the changes that ultimately lead to ischemia, tissue injury, and fetal loss,” they wrote.

The deposition of complement activation products in patients with antibodies occurs because the protection provided by complement regulatory proteins is overwhelmed by antibodies. This deposition is likely due to increased activation of the complement system, rather than a depletion or inactivation of the complement regulatory proteins.

DESTIN, FLA. — Pregnant women with antiphospholipid syndrome need anticoagulation throughout pregnancy and for at least 6 weeks post partum, Dr. Ann Parke said at the annual Rheumatology on the Beach.

Low-dose aspirin can also be added to anticoagulant therapy. The antimalarial drug hydroxychloroquine should be continued in those with systemic lupus erythematosus (SLE), said Dr. Parke, professor of medicine at the University of Connecticut, Farmington.

Discontinuing hydroxychloroquine will put the patient at risk of an SLE flare, putting the pregnancy and patient in jeopardy. SLE flares, as well as pregnancy, are among the known “second hit” phenomena that can trigger thrombosis in patients with antiphospholipid antibodies, Dr. Parke said. (See related story.)

Furthermore, data suggest that this drug—which reverses platelet activation induced by human IgG anticardiolipin, and which has been shown to reduce thrombosis size in antiphospholipid animal models—is safe in pregnancy, she added.

Other treatments used in pregnant patients with antiphospholipid syndrome (APS) include corticosteroids and intravenous immunoglobulin. IVIG is expensive, and there is no clear evidence of benefit, said Dr. Parke. Corticosteroids, which lost favor because of associated increases in maternal morbidity and risk of preterm birth, may regain ground in light of new evidence that complement activation plays a role in antiphospholipid antibody-associated placental injury. (See box.)

Pregnant patients with APS should be monitored closely for signs of placental insufficiency, HELLP (hemolytic anemia, elevated liver enzymes, low platelet count) syndrome, and toxemia, she said, noting more than 80% of women with APS experience at least one fetal death; in fact, the specificity of fetal death for the presence of antiphospholipid antibodies is 76%.

As for the treatment of pregnant patients with antiphospholipid antibodies who don't meet the criteria for antiphospholipid syndrome, a literature review published last year (JAMA 2006;295:1050–7) suggested asymptomatic patients and those who have experienced a single fetal loss before 10 weeks' gestation should receive no treatment, and that those with recurrent losses but no clinical thrombotic event should receive low-dose aspirin and prophylactic heparin throughout pregnancy and for a minimum of 6 weeks post partum. (Dr. Parke said she suspects this will be increased to 4 months because recent evidence suggests the risk of thrombosis is high throughout the first postpartum year.)

Like her recommendations for those with APS, the findings from the literature review supported the use of low-dose aspirin and therapeutic heparin throughout pregnancy, plus long-term warfarin post partum in those with recurrent losses and a clinical thrombotic event (who thus meet current Sapporo Criteria for APS).

Warfarin use, however, depends on the nature of the antibodies present. Lupus anticoagulant is more likely to be associated with clinical events than is anticardiolipin, for example. Titer, persistence, and isotype of the antibodies; the number of positive antibodies; and the presence of additional risk factors, such as SLE, also play into the decision to prescribe warfarin. A number of studies have shown moderate warfarin doses are as effective as and safer than high doses in such patients. It remains unclear whether it is necessary to use warfarin in patients with antibodies who have a clinical thrombotic event triggered by second hit phenomenon, she said.

Other issues not adequately addressed include therapy for patients with antibodies but no clinical event, optimal therapy for noncerebral artery thrombosis, managing patients who have recurrence despite adequate international normalized ratios, and therapy in women with antibodies and recurrent fetal losses.

Studies Probe APLA, Complement Links

Animal studies show preventing complement activation is essential in pregnancy, and deficiencies of certain complement components in the presence of antiphospholipid antibodies (APLA) prevent fetal death. Inhibitors of these components are protective in antibody-exposed mice. A study of human placentas suggests complement activation plays a role in APLA-associated placental injury, Dr. Parke said.

Using immunohistochemical methods to identify deposition of complement activation products in the placentas of 47 full-term viable pregnancies in women with APLA and 23 control placentas, investigators showed those with antibodies had more deposition of complement components C4d, C3b, and C5b-9 in the villous trophoblast cytoplasm and the extravillous trophoblast of the basal plate, and deposition of C4d in the trophoblastic cell and basement membrane (Am. J. Obstet. Gynecol. 2007;196:167).

Immunoreactivity was stronger for the C4d protein in all three areas in the APLA placentas and for the C3b protein in the villous trophoblast cytoplasm in the APLA placentas, versus controls. A link was seen between pathologic lesions and the deposition of C4d in the trophoblast cytoplasm and cellular and basement membranes.

In light of prior data showing trophoblastic cell membranes are targets for APLA, and the finding that placental lesions in women with these antibodies are tied to malperfusion, it seems “proinflammatory factors that stimulate complement activation may precede the changes that ultimately lead to ischemia, tissue injury, and fetal loss,” they wrote.

The deposition of complement activation products in patients with antibodies occurs because the protection provided by complement regulatory proteins is overwhelmed by antibodies. This deposition is likely due to increased activation of the complement system, rather than a depletion or inactivation of the complement regulatory proteins.

DESTIN, FLA. — A persistent Gottron's rash is the best predictor of childhood dermatomyositis disease course, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

In a study of 81 children with this inflammatory connective tissue disease who were followed for at least 3 years, a treatment-resistant Gottron's rash at 3 months following diagnosis predicted significantly longer time to remission (84 months vs. 41 months in those with no rash at 3 months).

At 6 months, predictors of remission included persistent Gottron's rash (hazard ratio 0.5) and nail fold abnormalities (hazard ratio 0.6), and at 12 months, a combination of Gottron's rash and weakness strongly predicted a chronic disease course, said Dr. Feldman, professor at the University of Toronto.

Gastrointestinal symptoms at diagnosis predicted significantly shorter time to remission (34 months vs. 84 months). The reason for this remains unclear, but in a poster on this data presented at the annual meeting of the American College of Rheumatology in November, Dr. Feldman and his colleagues noted that specific treatment regimens used early in the course of the disease may have contributed to the findings, which will be analyzed further.

The study also showed 60% of patients had chronic disease, 37% had monocyclic disease, and 3% had polycyclic disease, suggesting in children, remission is permanent. “When it's gone, it's almost always gone, and we can tell our patients that they are likely to stay in remission for the rest of their lives,” Dr. Feldman said.

Patients in the study had a mean age of nearly 8 years and a mean follow-up of nearly 6 years. The median time to remission was 4 years. About half remitted within 41/2 years, and at 10 years, about one-third still had chronic active disease, he said.

DESTIN, FLA. — A persistent Gottron's rash is the best predictor of childhood dermatomyositis disease course, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

In a study of 81 children with this inflammatory connective tissue disease who were followed for at least 3 years, a treatment-resistant Gottron's rash at 3 months following diagnosis predicted significantly longer time to remission (84 months vs. 41 months in those with no rash at 3 months).

At 6 months, predictors of remission included persistent Gottron's rash (hazard ratio 0.5) and nail fold abnormalities (hazard ratio 0.6), and at 12 months, a combination of Gottron's rash and weakness strongly predicted a chronic disease course, said Dr. Feldman, professor at the University of Toronto.

Gastrointestinal symptoms at diagnosis predicted significantly shorter time to remission (34 months vs. 84 months). The reason for this remains unclear, but in a poster on this data presented at the annual meeting of the American College of Rheumatology in November, Dr. Feldman and his colleagues noted that specific treatment regimens used early in the course of the disease may have contributed to the findings, which will be analyzed further.

The study also showed 60% of patients had chronic disease, 37% had monocyclic disease, and 3% had polycyclic disease, suggesting in children, remission is permanent. “When it's gone, it's almost always gone, and we can tell our patients that they are likely to stay in remission for the rest of their lives,” Dr. Feldman said.

Patients in the study had a mean age of nearly 8 years and a mean follow-up of nearly 6 years. The median time to remission was 4 years. About half remitted within 41/2 years, and at 10 years, about one-third still had chronic active disease, he said.

DESTIN, FLA. — A persistent Gottron's rash is the best predictor of childhood dermatomyositis disease course, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

In a study of 81 children with this inflammatory connective tissue disease who were followed for at least 3 years, a treatment-resistant Gottron's rash at 3 months following diagnosis predicted significantly longer time to remission (84 months vs. 41 months in those with no rash at 3 months).

At 6 months, predictors of remission included persistent Gottron's rash (hazard ratio 0.5) and nail fold abnormalities (hazard ratio 0.6), and at 12 months, a combination of Gottron's rash and weakness strongly predicted a chronic disease course, said Dr. Feldman, professor at the University of Toronto.

Gastrointestinal symptoms at diagnosis predicted significantly shorter time to remission (34 months vs. 84 months). The reason for this remains unclear, but in a poster on this data presented at the annual meeting of the American College of Rheumatology in November, Dr. Feldman and his colleagues noted that specific treatment regimens used early in the course of the disease may have contributed to the findings, which will be analyzed further.

The study also showed 60% of patients had chronic disease, 37% had monocyclic disease, and 3% had polycyclic disease, suggesting in children, remission is permanent. “When it's gone, it's almost always gone, and we can tell our patients that they are likely to stay in remission for the rest of their lives,” Dr. Feldman said.

Patients in the study had a mean age of nearly 8 years and a mean follow-up of nearly 6 years. The median time to remission was 4 years. About half remitted within 41/2 years, and at 10 years, about one-third still had chronic active disease, he said.

DESTIN, FLA. — Dermatomyositis is by far the most common form of idiopathic myositis in children, accounting for nearly 90% of cases, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

This differs from adult disease in that only about 14% of adult patients with myositis have dermatomyositis, with 11%–14% experiencing cancer-associated myositis, about 25% having overlap disease, and close to 50% having either polymyositis or inclusion body myositis.

Cancer-associated dermatomyositis almost never occurs in children, thus a work-up for underlying malignancy is not necessary in this patient population, said Dr. Feldman, a professor at the University of Toronto.

The disease in children can range from mild to severe, and a papulosquamous heliotrope rash, usually on the extensor surfaces, is typical. Such a rash that occurs over the knuckles is known as Gottron's rash, and this is also characteristic of the disease.

Calcinosis is a common finding in children, unlike in adults. It can be superficial or deep; in a third of patients, scarring lesions can be seen as long-term sequelae of the disease.

Systemic manifestations and cardiac disease can also occur in children with dermatomyositis, but both are more common in adults than in children.

Another finding is periungual erythema resulting from capillary nailfold changes. In fact, the underlying pathology in the disease is capillary vasculopathy and it has been shown that changes in the capillaries in the nailfold reflect disease activity changes, Dr. Feldman said.

In one study, Dr. Feldman showed capillary density in the nailfold as measured by capilloscopy strongly reflected disease activity in 42 children; as disease activity rose, capillary density was reduced, he said. The relationship did not extend to muscle damage.

Disability can be measured using the Childhood Health Assessment Questionnaire. Originally developed for evaluating disability in children with arthritis, this tool has also been shown to be a valid and responsive tool in these patients, he said.

Another tool useful for following dermatomyositis patients is the Childhood Myositis Assessment Scale. The 14-point scale is an occupational measure of muscle function that uses a series of maneuvers standardized by a group of experts in the field to measure patient ability.

“It turns out this is a phenomenal tool to follow children with myositis,” Dr. Feldman said, noting that he uses it for every patient at every visit.

The availability of such tools led to the idea that it might be possible to predict outcomes in children with dermatomyositis, he added.

In adults, dermatomyositis has been shown to be progressive, with increasing disability each year. In one study, mortality in adults was high at 25% over a 7- to 8-year period.

In children, however, the outcomes are much better. In one study of 65 patients followed for about 7 years, only 1 died, physical function was excellent in most of the children, and educational and vocational achievements were also excellent. A substantial number of patients, however, remained chronically affected, with more than a third continuing to require medication for the disease at 7 years, including 15% who remained on prednisone.

In a recent study (see related story) of an inception cohort of patients followed using the validated tools, Dr. Feldman and his colleagues showed that there are factors—such as persistent Gottron's rash—that can predict disease course and chronicity. Patients with a persistent rash at 3 months had increased time to remission, and those with persistent rash at 12 months were significantly more likely to have a chronic disease course, he said.

The study also showed that in children, disease remission was almost always permanent.

DESTIN, FLA. — Dermatomyositis is by far the most common form of idiopathic myositis in children, accounting for nearly 90% of cases, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

This differs from adult disease in that only about 14% of adult patients with myositis have dermatomyositis, with 11%–14% experiencing cancer-associated myositis, about 25% having overlap disease, and close to 50% having either polymyositis or inclusion body myositis.

Cancer-associated dermatomyositis almost never occurs in children, thus a work-up for underlying malignancy is not necessary in this patient population, said Dr. Feldman, a professor at the University of Toronto.

The disease in children can range from mild to severe, and a papulosquamous heliotrope rash, usually on the extensor surfaces, is typical. Such a rash that occurs over the knuckles is known as Gottron's rash, and this is also characteristic of the disease.

Calcinosis is a common finding in children, unlike in adults. It can be superficial or deep; in a third of patients, scarring lesions can be seen as long-term sequelae of the disease.

Systemic manifestations and cardiac disease can also occur in children with dermatomyositis, but both are more common in adults than in children.

Another finding is periungual erythema resulting from capillary nailfold changes. In fact, the underlying pathology in the disease is capillary vasculopathy and it has been shown that changes in the capillaries in the nailfold reflect disease activity changes, Dr. Feldman said.

In one study, Dr. Feldman showed capillary density in the nailfold as measured by capilloscopy strongly reflected disease activity in 42 children; as disease activity rose, capillary density was reduced, he said. The relationship did not extend to muscle damage.

Disability can be measured using the Childhood Health Assessment Questionnaire. Originally developed for evaluating disability in children with arthritis, this tool has also been shown to be a valid and responsive tool in these patients, he said.

Another tool useful for following dermatomyositis patients is the Childhood Myositis Assessment Scale. The 14-point scale is an occupational measure of muscle function that uses a series of maneuvers standardized by a group of experts in the field to measure patient ability.

“It turns out this is a phenomenal tool to follow children with myositis,” Dr. Feldman said, noting that he uses it for every patient at every visit.

The availability of such tools led to the idea that it might be possible to predict outcomes in children with dermatomyositis, he added.

In adults, dermatomyositis has been shown to be progressive, with increasing disability each year. In one study, mortality in adults was high at 25% over a 7- to 8-year period.

In children, however, the outcomes are much better. In one study of 65 patients followed for about 7 years, only 1 died, physical function was excellent in most of the children, and educational and vocational achievements were also excellent. A substantial number of patients, however, remained chronically affected, with more than a third continuing to require medication for the disease at 7 years, including 15% who remained on prednisone.

In a recent study (see related story) of an inception cohort of patients followed using the validated tools, Dr. Feldman and his colleagues showed that there are factors—such as persistent Gottron's rash—that can predict disease course and chronicity. Patients with a persistent rash at 3 months had increased time to remission, and those with persistent rash at 12 months were significantly more likely to have a chronic disease course, he said.

The study also showed that in children, disease remission was almost always permanent.

DESTIN, FLA. — Dermatomyositis is by far the most common form of idiopathic myositis in children, accounting for nearly 90% of cases, Dr. Brian Feldman said at the annual Rheumatology on the Beach.

This differs from adult disease in that only about 14% of adult patients with myositis have dermatomyositis, with 11%–14% experiencing cancer-associated myositis, about 25% having overlap disease, and close to 50% having either polymyositis or inclusion body myositis.

Cancer-associated dermatomyositis almost never occurs in children, thus a work-up for underlying malignancy is not necessary in this patient population, said Dr. Feldman, a professor at the University of Toronto.

The disease in children can range from mild to severe, and a papulosquamous heliotrope rash, usually on the extensor surfaces, is typical. Such a rash that occurs over the knuckles is known as Gottron's rash, and this is also characteristic of the disease.

Calcinosis is a common finding in children, unlike in adults. It can be superficial or deep; in a third of patients, scarring lesions can be seen as long-term sequelae of the disease.

Systemic manifestations and cardiac disease can also occur in children with dermatomyositis, but both are more common in adults than in children.

Another finding is periungual erythema resulting from capillary nailfold changes. In fact, the underlying pathology in the disease is capillary vasculopathy and it has been shown that changes in the capillaries in the nailfold reflect disease activity changes, Dr. Feldman said.

In one study, Dr. Feldman showed capillary density in the nailfold as measured by capilloscopy strongly reflected disease activity in 42 children; as disease activity rose, capillary density was reduced, he said. The relationship did not extend to muscle damage.

Disability can be measured using the Childhood Health Assessment Questionnaire. Originally developed for evaluating disability in children with arthritis, this tool has also been shown to be a valid and responsive tool in these patients, he said.

Another tool useful for following dermatomyositis patients is the Childhood Myositis Assessment Scale. The 14-point scale is an occupational measure of muscle function that uses a series of maneuvers standardized by a group of experts in the field to measure patient ability.

“It turns out this is a phenomenal tool to follow children with myositis,” Dr. Feldman said, noting that he uses it for every patient at every visit.

The availability of such tools led to the idea that it might be possible to predict outcomes in children with dermatomyositis, he added.

In adults, dermatomyositis has been shown to be progressive, with increasing disability each year. In one study, mortality in adults was high at 25% over a 7- to 8-year period.

In children, however, the outcomes are much better. In one study of 65 patients followed for about 7 years, only 1 died, physical function was excellent in most of the children, and educational and vocational achievements were also excellent. A substantial number of patients, however, remained chronically affected, with more than a third continuing to require medication for the disease at 7 years, including 15% who remained on prednisone.

In a recent study (see related story) of an inception cohort of patients followed using the validated tools, Dr. Feldman and his colleagues showed that there are factors—such as persistent Gottron's rash—that can predict disease course and chronicity. Patients with a persistent rash at 3 months had increased time to remission, and those with persistent rash at 12 months were significantly more likely to have a chronic disease course, he said.

The study also showed that in children, disease remission was almost always permanent.

TAMPA — About a third of diabetic patients with foot neuropathy have pain and loss of sensation resulting from nerve compression, and these patients probably will benefit substantially from nerve decompression surgery, Dr. A. Lee Dellon said at the annual meeting of the Wound Healing Society.

Data are increasingly demonstrating the benefits of this surgery—which is much like the surgery used to treat carpal tunnel syndrome—in select patients with foot neuropathy, said Dr. Dellon, of the division of plastic surgery at Johns Hopkins University, Baltimore.

In a series of 765 patients, the ulceration rate following successful tissue reinnervation in patients with neuropathy but no prior ulceration was 0.5%, compared with an expected rate of 15% in nonsurgery patients. The rate in those with a previous ulcer that healed was 5%, compared with an expected rate of 50%. No amputations were required in the reinnervated patients.

Results for Dr. Dellon's first series of patients treated with nerve decompression for foot neuropathy were published in 1992. The site of compression was identified in the patients by a Tinel's sign, which in a more recent study was shown to have a positive predictive value of 92% for nerve compression, he noted.

In the 1992 series, outcomes in individual nerves were good to excellent in 80% of patients at an average follow-up of 2.5 years. In one recent study, restoration of sensation in patients who underwent nerve decompression for foot neuropathy was associated with a 3% ulceration recurrence rate, 80% improvement in pain, and 80% recovery of sensibility, with no new ulcers or amputations.

Patients who undergo nerve decompression surgery often have immediate results. One such patient told Dr. Dellon that her surgery, which had occurred just 24 hours prior, had already improved her pain by 50% and restored sensation to her toes.

“When nerve fibers haven't totally died, they can have a reversible ischemic block. When the nerve is decompressed and blood flows into the nerve, the nerve can conduct a transmitted impulse again,” he explained, adding that about half of patients will “actually wake up with this reaction.”

It takes about a year, however, for the nerve to regenerate out to the toes, Dr. Dellon noted. Ideal nerve decompression candidates are those who have neuropathy symptoms and documented neuropathy, tight glycemic control, failure to respond to neuropathic pain medications, good circulation, no edema, and a positive Tinel's sign, he said at the meeting, which was held in conjunction with a symposium on advanced wound care.

TAMPA — About a third of diabetic patients with foot neuropathy have pain and loss of sensation resulting from nerve compression, and these patients probably will benefit substantially from nerve decompression surgery, Dr. A. Lee Dellon said at the annual meeting of the Wound Healing Society.

Data are increasingly demonstrating the benefits of this surgery—which is much like the surgery used to treat carpal tunnel syndrome—in select patients with foot neuropathy, said Dr. Dellon, of the division of plastic surgery at Johns Hopkins University, Baltimore.

In a series of 765 patients, the ulceration rate following successful tissue reinnervation in patients with neuropathy but no prior ulceration was 0.5%, compared with an expected rate of 15% in nonsurgery patients. The rate in those with a previous ulcer that healed was 5%, compared with an expected rate of 50%. No amputations were required in the reinnervated patients.

Results for Dr. Dellon's first series of patients treated with nerve decompression for foot neuropathy were published in 1992. The site of compression was identified in the patients by a Tinel's sign, which in a more recent study was shown to have a positive predictive value of 92% for nerve compression, he noted.

In the 1992 series, outcomes in individual nerves were good to excellent in 80% of patients at an average follow-up of 2.5 years. In one recent study, restoration of sensation in patients who underwent nerve decompression for foot neuropathy was associated with a 3% ulceration recurrence rate, 80% improvement in pain, and 80% recovery of sensibility, with no new ulcers or amputations.

Patients who undergo nerve decompression surgery often have immediate results. One such patient told Dr. Dellon that her surgery, which had occurred just 24 hours prior, had already improved her pain by 50% and restored sensation to her toes.

“When nerve fibers haven't totally died, they can have a reversible ischemic block. When the nerve is decompressed and blood flows into the nerve, the nerve can conduct a transmitted impulse again,” he explained, adding that about half of patients will “actually wake up with this reaction.”

It takes about a year, however, for the nerve to regenerate out to the toes, Dr. Dellon noted. Ideal nerve decompression candidates are those who have neuropathy symptoms and documented neuropathy, tight glycemic control, failure to respond to neuropathic pain medications, good circulation, no edema, and a positive Tinel's sign, he said at the meeting, which was held in conjunction with a symposium on advanced wound care.

TAMPA — About a third of diabetic patients with foot neuropathy have pain and loss of sensation resulting from nerve compression, and these patients probably will benefit substantially from nerve decompression surgery, Dr. A. Lee Dellon said at the annual meeting of the Wound Healing Society.

Data are increasingly demonstrating the benefits of this surgery—which is much like the surgery used to treat carpal tunnel syndrome—in select patients with foot neuropathy, said Dr. Dellon, of the division of plastic surgery at Johns Hopkins University, Baltimore.

In a series of 765 patients, the ulceration rate following successful tissue reinnervation in patients with neuropathy but no prior ulceration was 0.5%, compared with an expected rate of 15% in nonsurgery patients. The rate in those with a previous ulcer that healed was 5%, compared with an expected rate of 50%. No amputations were required in the reinnervated patients.

Results for Dr. Dellon's first series of patients treated with nerve decompression for foot neuropathy were published in 1992. The site of compression was identified in the patients by a Tinel's sign, which in a more recent study was shown to have a positive predictive value of 92% for nerve compression, he noted.

In the 1992 series, outcomes in individual nerves were good to excellent in 80% of patients at an average follow-up of 2.5 years. In one recent study, restoration of sensation in patients who underwent nerve decompression for foot neuropathy was associated with a 3% ulceration recurrence rate, 80% improvement in pain, and 80% recovery of sensibility, with no new ulcers or amputations.

Patients who undergo nerve decompression surgery often have immediate results. One such patient told Dr. Dellon that her surgery, which had occurred just 24 hours prior, had already improved her pain by 50% and restored sensation to her toes.

“When nerve fibers haven't totally died, they can have a reversible ischemic block. When the nerve is decompressed and blood flows into the nerve, the nerve can conduct a transmitted impulse again,” he explained, adding that about half of patients will “actually wake up with this reaction.”

It takes about a year, however, for the nerve to regenerate out to the toes, Dr. Dellon noted. Ideal nerve decompression candidates are those who have neuropathy symptoms and documented neuropathy, tight glycemic control, failure to respond to neuropathic pain medications, good circulation, no edema, and a positive Tinel's sign, he said at the meeting, which was held in conjunction with a symposium on advanced wound care.

MIAMI BEACH – Comorbid psychiatric conditions are common in patients with headache disorders, and can adversely affect the prognosis in patients with such disorders, Alvin E. Lake III, Ph.D., said at a symposium sponsored by the American Headache Society.

However, combined behavioral and drug therapy as well as patient education have been shown to improve outcomes, said Dr. Lake, director of the behavioral medicine division at the Michigan Headache and Neurological Institute, Ann Arbor.

Studies suggest that close to 50% of patients with chronic daily headache have an anxiety and/or mood disorder. In those with medication overuse headaches, the prevalence of mood and anxiety disorders appears to be even higher at 68%, according to one study.

Headache patients with psychiatric disorders also appear to have poorer long-term outcomes than do those with no psychiatric disorder. In one study, 57% of patients with multiple psychiatric disorders had worsening of their headaches over an 8-year period, compared with 7% of those with no psychiatric disorder. In addition, 29% of those with multiple psychiatric disorders experienced improvement, compared with 53% of those with no psychiatric disorder.

It appears that in most cases, the psychiatric disorders preceded the headache disorders. In a study of 41 patients with medication overuse headaches and comorbid psychiatric disorders, the psychiatric disorder preceded the headaches in 76% of those with a major depressive episode, 79% of those with panic disorder, 80% of those with generalized anxiety disorder, 89% of those with substance abuse disorder, and 100% of those with social phobia, Dr. Lake noted.

In addition to mood disorders, which have a genetic component, psychological factors, such as anticipatory fear of pain, and psychosocial factors, such as family and work pressures and a need to function, can drive excessive use of preemptive treatment, which in turn can lead to headache chronicity, he explained.

In one study of headache patients, the use of analgesics at initial assessment was associated with a relative risk of 19.6 for chronic daily headaches at 11-year follow-up, compared with a relative risk of 3.1 in those without analgesic overuse. Daily or weekly analgesic use also elevated the risk for chronic pain; in those who used analgesics more than 15 days per month, the relative risk of chronic migraine was 13.3 and the relative risk of nonmigraine headache was 6.2, compared with those without analgesic overuse.

Differential attention to the headache pain has been shown to modulate the subjective experience of pain, Dr. Lake said.

For example, attention to pain location increases responses in the somatosensory cortex, while attention to the unpleasantness of the pain increases responses in the limbic system.

Distraction, such as activities that divert attention away from the pain, can lower pain intensity and increase brain stem periaqueductal gray activation, which has been shown to predict changes in perceived pain intensity.

Thus, behavioral therapy is useful in this patient population. Several studies demonstrate improved outcomes with combined treatment.

For example, in one study of patients with chronic tension headaches, 64% of patients who received tricyclic antidepressants as well as stress management training had improvement at 8 months, compared with 29% of placebo patients, 38% of those on tricyclic antidepressants alone, and 35% who received stress management training alone.

In another study of patients with medication overuse headaches, headache days per month were reduced at 3 years' follow-up from 30 to 11 days in patients who received inpatient pharmacologic therapy and biofeedback-assisted relaxation training, compared with a reduction from 30 to 18 days in those who received only inpatient pharmacologic therapy.

Analgesic doses per month were reduced from 59 to 4 doses in the combination treatment group, compared with a reduction from 59 to 20 doses in those with preventive medication treatment alone. The relapse rate to medication overuse headaches was 13% in the combination treatment group, compared with 42% in the medication-only group.

Patient education has also been shown to be of benefit, Dr. Lake said.

A study published in May 2006 showed that patients who attended three 90-minute educational sessions taught by intensively trained lay migraineurs was useful for improving outcomes and reducing analgesic overuse (Headache 2006;46:726–31).

Of 100 consecutive migraine patients who received routine medical management and were randomly assigned to attend or not attend the classes–which offered information on migraine pathogenesis, management, and risks of rebound–those who attended had a significantly greater reduction in mean migraine disability assessment scores (reduction of 24 vs. 14 points) at 6 months.

They also had fewer headache days per month, less headache-related dysfunction, less abortive medication use, less analgesic overuse, and fewer headache-related phone calls and unscheduled visits to doctors, Dr. Lake said.

MIAMI BEACH – Comorbid psychiatric conditions are common in patients with headache disorders, and can adversely affect the prognosis in patients with such disorders, Alvin E. Lake III, Ph.D., said at a symposium sponsored by the American Headache Society.

However, combined behavioral and drug therapy as well as patient education have been shown to improve outcomes, said Dr. Lake, director of the behavioral medicine division at the Michigan Headache and Neurological Institute, Ann Arbor.

Studies suggest that close to 50% of patients with chronic daily headache have an anxiety and/or mood disorder. In those with medication overuse headaches, the prevalence of mood and anxiety disorders appears to be even higher at 68%, according to one study.

Headache patients with psychiatric disorders also appear to have poorer long-term outcomes than do those with no psychiatric disorder. In one study, 57% of patients with multiple psychiatric disorders had worsening of their headaches over an 8-year period, compared with 7% of those with no psychiatric disorder. In addition, 29% of those with multiple psychiatric disorders experienced improvement, compared with 53% of those with no psychiatric disorder.

It appears that in most cases, the psychiatric disorders preceded the headache disorders. In a study of 41 patients with medication overuse headaches and comorbid psychiatric disorders, the psychiatric disorder preceded the headaches in 76% of those with a major depressive episode, 79% of those with panic disorder, 80% of those with generalized anxiety disorder, 89% of those with substance abuse disorder, and 100% of those with social phobia, Dr. Lake noted.

In addition to mood disorders, which have a genetic component, psychological factors, such as anticipatory fear of pain, and psychosocial factors, such as family and work pressures and a need to function, can drive excessive use of preemptive treatment, which in turn can lead to headache chronicity, he explained.

In one study of headache patients, the use of analgesics at initial assessment was associated with a relative risk of 19.6 for chronic daily headaches at 11-year follow-up, compared with a relative risk of 3.1 in those without analgesic overuse. Daily or weekly analgesic use also elevated the risk for chronic pain; in those who used analgesics more than 15 days per month, the relative risk of chronic migraine was 13.3 and the relative risk of nonmigraine headache was 6.2, compared with those without analgesic overuse.

Differential attention to the headache pain has been shown to modulate the subjective experience of pain, Dr. Lake said.

For example, attention to pain location increases responses in the somatosensory cortex, while attention to the unpleasantness of the pain increases responses in the limbic system.

Distraction, such as activities that divert attention away from the pain, can lower pain intensity and increase brain stem periaqueductal gray activation, which has been shown to predict changes in perceived pain intensity.

Thus, behavioral therapy is useful in this patient population. Several studies demonstrate improved outcomes with combined treatment.

For example, in one study of patients with chronic tension headaches, 64% of patients who received tricyclic antidepressants as well as stress management training had improvement at 8 months, compared with 29% of placebo patients, 38% of those on tricyclic antidepressants alone, and 35% who received stress management training alone.

In another study of patients with medication overuse headaches, headache days per month were reduced at 3 years' follow-up from 30 to 11 days in patients who received inpatient pharmacologic therapy and biofeedback-assisted relaxation training, compared with a reduction from 30 to 18 days in those who received only inpatient pharmacologic therapy.

Analgesic doses per month were reduced from 59 to 4 doses in the combination treatment group, compared with a reduction from 59 to 20 doses in those with preventive medication treatment alone. The relapse rate to medication overuse headaches was 13% in the combination treatment group, compared with 42% in the medication-only group.

Patient education has also been shown to be of benefit, Dr. Lake said.

A study published in May 2006 showed that patients who attended three 90-minute educational sessions taught by intensively trained lay migraineurs was useful for improving outcomes and reducing analgesic overuse (Headache 2006;46:726–31).

Of 100 consecutive migraine patients who received routine medical management and were randomly assigned to attend or not attend the classes–which offered information on migraine pathogenesis, management, and risks of rebound–those who attended had a significantly greater reduction in mean migraine disability assessment scores (reduction of 24 vs. 14 points) at 6 months.

They also had fewer headache days per month, less headache-related dysfunction, less abortive medication use, less analgesic overuse, and fewer headache-related phone calls and unscheduled visits to doctors, Dr. Lake said.

MIAMI BEACH – Comorbid psychiatric conditions are common in patients with headache disorders, and can adversely affect the prognosis in patients with such disorders, Alvin E. Lake III, Ph.D., said at a symposium sponsored by the American Headache Society.

However, combined behavioral and drug therapy as well as patient education have been shown to improve outcomes, said Dr. Lake, director of the behavioral medicine division at the Michigan Headache and Neurological Institute, Ann Arbor.

Studies suggest that close to 50% of patients with chronic daily headache have an anxiety and/or mood disorder. In those with medication overuse headaches, the prevalence of mood and anxiety disorders appears to be even higher at 68%, according to one study.

Headache patients with psychiatric disorders also appear to have poorer long-term outcomes than do those with no psychiatric disorder. In one study, 57% of patients with multiple psychiatric disorders had worsening of their headaches over an 8-year period, compared with 7% of those with no psychiatric disorder. In addition, 29% of those with multiple psychiatric disorders experienced improvement, compared with 53% of those with no psychiatric disorder.

It appears that in most cases, the psychiatric disorders preceded the headache disorders. In a study of 41 patients with medication overuse headaches and comorbid psychiatric disorders, the psychiatric disorder preceded the headaches in 76% of those with a major depressive episode, 79% of those with panic disorder, 80% of those with generalized anxiety disorder, 89% of those with substance abuse disorder, and 100% of those with social phobia, Dr. Lake noted.

In addition to mood disorders, which have a genetic component, psychological factors, such as anticipatory fear of pain, and psychosocial factors, such as family and work pressures and a need to function, can drive excessive use of preemptive treatment, which in turn can lead to headache chronicity, he explained.

In one study of headache patients, the use of analgesics at initial assessment was associated with a relative risk of 19.6 for chronic daily headaches at 11-year follow-up, compared with a relative risk of 3.1 in those without analgesic overuse. Daily or weekly analgesic use also elevated the risk for chronic pain; in those who used analgesics more than 15 days per month, the relative risk of chronic migraine was 13.3 and the relative risk of nonmigraine headache was 6.2, compared with those without analgesic overuse.

Differential attention to the headache pain has been shown to modulate the subjective experience of pain, Dr. Lake said.

For example, attention to pain location increases responses in the somatosensory cortex, while attention to the unpleasantness of the pain increases responses in the limbic system.

Distraction, such as activities that divert attention away from the pain, can lower pain intensity and increase brain stem periaqueductal gray activation, which has been shown to predict changes in perceived pain intensity.

Thus, behavioral therapy is useful in this patient population. Several studies demonstrate improved outcomes with combined treatment.

For example, in one study of patients with chronic tension headaches, 64% of patients who received tricyclic antidepressants as well as stress management training had improvement at 8 months, compared with 29% of placebo patients, 38% of those on tricyclic antidepressants alone, and 35% who received stress management training alone.

In another study of patients with medication overuse headaches, headache days per month were reduced at 3 years' follow-up from 30 to 11 days in patients who received inpatient pharmacologic therapy and biofeedback-assisted relaxation training, compared with a reduction from 30 to 18 days in those who received only inpatient pharmacologic therapy.

Analgesic doses per month were reduced from 59 to 4 doses in the combination treatment group, compared with a reduction from 59 to 20 doses in those with preventive medication treatment alone. The relapse rate to medication overuse headaches was 13% in the combination treatment group, compared with 42% in the medication-only group.

Patient education has also been shown to be of benefit, Dr. Lake said.

A study published in May 2006 showed that patients who attended three 90-minute educational sessions taught by intensively trained lay migraineurs was useful for improving outcomes and reducing analgesic overuse (Headache 2006;46:726–31).

Of 100 consecutive migraine patients who received routine medical management and were randomly assigned to attend or not attend the classes–which offered information on migraine pathogenesis, management, and risks of rebound–those who attended had a significantly greater reduction in mean migraine disability assessment scores (reduction of 24 vs. 14 points) at 6 months.

They also had fewer headache days per month, less headache-related dysfunction, less abortive medication use, less analgesic overuse, and fewer headache-related phone calls and unscheduled visits to doctors, Dr. Lake said.

MIAMI BEACH — Up to 70% of women who experience migraines have exacerbations during menstruation, and another 7%–14% of female migraineurs experience only menstrually related migraines.

Reducing the drop in estrogen levels that occurs at menses—whether the drop is endogenous or exogenous—can help these women, Dr. Susan Hutchinson said at a symposium sponsored by the American Headache Society.

In women on oral contraceptives who experience migraine without aura, add-back estrogen delivered perimenstrually when cycling off the active pills may help prevent menstrual migraines. Add-back estrogen can also prevent the endogenous drop in ovarian estradiol production in women not using hormonal contraception who have menstrual migraines.

Physicians might consider using a 0.1-mg dose delivered via estradiol patch during the week of menses, said Dr. Hutchinson, a family physician and headache specialist in private practice in Irvine, Calif. Lower doses tend to be less effective for this purpose, she added.

Young female migraineurs who ask for oral contraception should be advised of the “one-third rule,” which is that about a third of migraineurs who start on oral contraception experience improvements, about a third have no change, and about a third have deterioration.

However, the best options in those with regular menses include low-dose (35 mcg of estrogen or less) monophasic birth control pills, or contraception delivered via a contraceptive ring.

For prevention in those who still have menstrual migraines, physicians should consider continuous monophasic contraception or continuous contraception via vaginal ring with estradiol add-back when cycling off.

However, Dr. Hutchinson warned that the World Health Organization and the American College of Obstetricians and Gynecologists consider estrogen-containing contraception contraindicated in women who have migraine with aura, because studies have shown an increased stroke risk in this population.

The risk is further increased in those who use hormonal contraceptives and who have other risk factors such as smoking, hypertension, and dense aura.

In women who experience aura only rarely, the benefits of estrogen-containing contraception may outweigh the risks, so treatment decisions should be made on an individual basis, Dr. Hutchinson said.

In those who experience aura with migraine, options include progesterone-only oral contraceptives, implants, or injections, and progesterone or copper IUDs.

All migraine patients whose hormonal status is altered should keep a journal or calendar tracking headaches to ensure appropriate treatment, she noted.

“Understanding the relationship between hormones and migraine is instrumental. … If we really want to help our women migraineurs, particularly the 60%–70% [whose headaches worsen] during the time of their period,” said Dr. Hutchinson.

Preventive Strategies MayWork for Nonresponders

Women with menstrual migraines who fail to respond adequately to hormonal manipulation may benefit from short-term or minipreventive treatment approaches, Dr. Hutchinson said.

For example, NSAIDs given for short periods or just before menstruation can be helpful for reducing frequency and/or severity of migraines. Triptans are also useful for menstrual migraines, but many women who use triptans express concerns about having to use so many for this indication, because menstrual migraines tend to last longer and have greater severity than other migraines. In these patients, combining hormone manipulation with triptan treatment may help.

Combining an NSAID and triptan can also be a useful approach; some patients report that this combination works even better and faster than triptans alone.

Magnesium has also been shown to have some preventive benefit when given at 400 mg during the luteal phase or daily (a simpler approach). Increasing the dose of daily preventive medications around the time of menstruation can also be useful, Dr. Hutchinson said.

MIAMI BEACH — Up to 70% of women who experience migraines have exacerbations during menstruation, and another 7%–14% of female migraineurs experience only menstrually related migraines.

Reducing the drop in estrogen levels that occurs at menses—whether the drop is endogenous or exogenous—can help these women, Dr. Susan Hutchinson said at a symposium sponsored by the American Headache Society.

In women on oral contraceptives who experience migraine without aura, add-back estrogen delivered perimenstrually when cycling off the active pills may help prevent menstrual migraines. Add-back estrogen can also prevent the endogenous drop in ovarian estradiol production in women not using hormonal contraception who have menstrual migraines.

Physicians might consider using a 0.1-mg dose delivered via estradiol patch during the week of menses, said Dr. Hutchinson, a family physician and headache specialist in private practice in Irvine, Calif. Lower doses tend to be less effective for this purpose, she added.

Young female migraineurs who ask for oral contraception should be advised of the “one-third rule,” which is that about a third of migraineurs who start on oral contraception experience improvements, about a third have no change, and about a third have deterioration.

However, the best options in those with regular menses include low-dose (35 mcg of estrogen or less) monophasic birth control pills, or contraception delivered via a contraceptive ring.

For prevention in those who still have menstrual migraines, physicians should consider continuous monophasic contraception or continuous contraception via vaginal ring with estradiol add-back when cycling off.

However, Dr. Hutchinson warned that the World Health Organization and the American College of Obstetricians and Gynecologists consider estrogen-containing contraception contraindicated in women who have migraine with aura, because studies have shown an increased stroke risk in this population.

The risk is further increased in those who use hormonal contraceptives and who have other risk factors such as smoking, hypertension, and dense aura.

In women who experience aura only rarely, the benefits of estrogen-containing contraception may outweigh the risks, so treatment decisions should be made on an individual basis, Dr. Hutchinson said.

In those who experience aura with migraine, options include progesterone-only oral contraceptives, implants, or injections, and progesterone or copper IUDs.

All migraine patients whose hormonal status is altered should keep a journal or calendar tracking headaches to ensure appropriate treatment, she noted.

“Understanding the relationship between hormones and migraine is instrumental. … If we really want to help our women migraineurs, particularly the 60%–70% [whose headaches worsen] during the time of their period,” said Dr. Hutchinson.

Preventive Strategies MayWork for Nonresponders

Women with menstrual migraines who fail to respond adequately to hormonal manipulation may benefit from short-term or minipreventive treatment approaches, Dr. Hutchinson said.

For example, NSAIDs given for short periods or just before menstruation can be helpful for reducing frequency and/or severity of migraines. Triptans are also useful for menstrual migraines, but many women who use triptans express concerns about having to use so many for this indication, because menstrual migraines tend to last longer and have greater severity than other migraines. In these patients, combining hormone manipulation with triptan treatment may help.

Combining an NSAID and triptan can also be a useful approach; some patients report that this combination works even better and faster than triptans alone.

Magnesium has also been shown to have some preventive benefit when given at 400 mg during the luteal phase or daily (a simpler approach). Increasing the dose of daily preventive medications around the time of menstruation can also be useful, Dr. Hutchinson said.

MIAMI BEACH — Up to 70% of women who experience migraines have exacerbations during menstruation, and another 7%–14% of female migraineurs experience only menstrually related migraines.

Reducing the drop in estrogen levels that occurs at menses—whether the drop is endogenous or exogenous—can help these women, Dr. Susan Hutchinson said at a symposium sponsored by the American Headache Society.

In women on oral contraceptives who experience migraine without aura, add-back estrogen delivered perimenstrually when cycling off the active pills may help prevent menstrual migraines. Add-back estrogen can also prevent the endogenous drop in ovarian estradiol production in women not using hormonal contraception who have menstrual migraines.

Physicians might consider using a 0.1-mg dose delivered via estradiol patch during the week of menses, said Dr. Hutchinson, a family physician and headache specialist in private practice in Irvine, Calif. Lower doses tend to be less effective for this purpose, she added.

Young female migraineurs who ask for oral contraception should be advised of the “one-third rule,” which is that about a third of migraineurs who start on oral contraception experience improvements, about a third have no change, and about a third have deterioration.

However, the best options in those with regular menses include low-dose (35 mcg of estrogen or less) monophasic birth control pills, or contraception delivered via a contraceptive ring.

For prevention in those who still have menstrual migraines, physicians should consider continuous monophasic contraception or continuous contraception via vaginal ring with estradiol add-back when cycling off.

However, Dr. Hutchinson warned that the World Health Organization and the American College of Obstetricians and Gynecologists consider estrogen-containing contraception contraindicated in women who have migraine with aura, because studies have shown an increased stroke risk in this population.

The risk is further increased in those who use hormonal contraceptives and who have other risk factors such as smoking, hypertension, and dense aura.

In women who experience aura only rarely, the benefits of estrogen-containing contraception may outweigh the risks, so treatment decisions should be made on an individual basis, Dr. Hutchinson said.

In those who experience aura with migraine, options include progesterone-only oral contraceptives, implants, or injections, and progesterone or copper IUDs.

All migraine patients whose hormonal status is altered should keep a journal or calendar tracking headaches to ensure appropriate treatment, she noted.

“Understanding the relationship between hormones and migraine is instrumental. … If we really want to help our women migraineurs, particularly the 60%–70% [whose headaches worsen] during the time of their period,” said Dr. Hutchinson.

Preventive Strategies MayWork for Nonresponders

Women with menstrual migraines who fail to respond adequately to hormonal manipulation may benefit from short-term or minipreventive treatment approaches, Dr. Hutchinson said.

For example, NSAIDs given for short periods or just before menstruation can be helpful for reducing frequency and/or severity of migraines. Triptans are also useful for menstrual migraines, but many women who use triptans express concerns about having to use so many for this indication, because menstrual migraines tend to last longer and have greater severity than other migraines. In these patients, combining hormone manipulation with triptan treatment may help.

Combining an NSAID and triptan can also be a useful approach; some patients report that this combination works even better and faster than triptans alone.

Magnesium has also been shown to have some preventive benefit when given at 400 mg during the luteal phase or daily (a simpler approach). Increasing the dose of daily preventive medications around the time of menstruation can also be useful, Dr. Hutchinson said.

MIAMI BEACH — Transjugular intrahepatic portosystemic shunt placement remains an effective treatment for ascites in select patients, Dr. Florence Wong said at a meeting on hepatobiliary disease sponsored by the University of Miami.

The treatment, commonly known as TIPS, has been shown to significantly improve urinary sodium excretion by eliminating portal hypertension, explained Dr. Wong, of the University of Toronto.

In studies of TIPS in patients with this complication of cirrhosis, urinary sodium excretion has been shown to improve gradually over 14 months without the use of diuretic therapy, she noted.

Additionally, five randomized controlled trials comparing TIPS with repeat large-volume paracentesis showed that TIPS was better for controlling ascites.

A recent meta-analysis using individual patient data points shows a survival advantage for TIPS, compared with large-volume paracentesis, said Dr. Wong, who has submitted the meta-analysis data for publication.

But TIPS is not appropriate for all patients. The studies show that outcomes are worse with increasing age and with cardiac or renal dysfunction at baseline. Renal excretion of sodium tends to worsen with increasing age and baseline renal dysfunction, as well as in those with preexisting cardiac or coronary disease, she explained.

Furthermore, patients with a history of hepatic encephalopathy tend to have worsening of the condition following TIPS.

“We also now know that in those with more severe liver disease, with shunting the liver becomes mildly ischemic, and by placing TIPS, we may actually precipitate liver failure,” Dr. Wong said.

TIPS can be considered in those under age 65 with normal cardiac and renal function, and without a history of hepatic encephalopathy or severe liver disease, she said.

MIAMI BEACH — Transjugular intrahepatic portosystemic shunt placement remains an effective treatment for ascites in select patients, Dr. Florence Wong said at a meeting on hepatobiliary disease sponsored by the University of Miami.

The treatment, commonly known as TIPS, has been shown to significantly improve urinary sodium excretion by eliminating portal hypertension, explained Dr. Wong, of the University of Toronto.

In studies of TIPS in patients with this complication of cirrhosis, urinary sodium excretion has been shown to improve gradually over 14 months without the use of diuretic therapy, she noted.

Additionally, five randomized controlled trials comparing TIPS with repeat large-volume paracentesis showed that TIPS was better for controlling ascites.

A recent meta-analysis using individual patient data points shows a survival advantage for TIPS, compared with large-volume paracentesis, said Dr. Wong, who has submitted the meta-analysis data for publication.

But TIPS is not appropriate for all patients. The studies show that outcomes are worse with increasing age and with cardiac or renal dysfunction at baseline. Renal excretion of sodium tends to worsen with increasing age and baseline renal dysfunction, as well as in those with preexisting cardiac or coronary disease, she explained.

Furthermore, patients with a history of hepatic encephalopathy tend to have worsening of the condition following TIPS.

“We also now know that in those with more severe liver disease, with shunting the liver becomes mildly ischemic, and by placing TIPS, we may actually precipitate liver failure,” Dr. Wong said.

TIPS can be considered in those under age 65 with normal cardiac and renal function, and without a history of hepatic encephalopathy or severe liver disease, she said.

MIAMI BEACH — Transjugular intrahepatic portosystemic shunt placement remains an effective treatment for ascites in select patients, Dr. Florence Wong said at a meeting on hepatobiliary disease sponsored by the University of Miami.

The treatment, commonly known as TIPS, has been shown to significantly improve urinary sodium excretion by eliminating portal hypertension, explained Dr. Wong, of the University of Toronto.

In studies of TIPS in patients with this complication of cirrhosis, urinary sodium excretion has been shown to improve gradually over 14 months without the use of diuretic therapy, she noted.

Additionally, five randomized controlled trials comparing TIPS with repeat large-volume paracentesis showed that TIPS was better for controlling ascites.

A recent meta-analysis using individual patient data points shows a survival advantage for TIPS, compared with large-volume paracentesis, said Dr. Wong, who has submitted the meta-analysis data for publication.

But TIPS is not appropriate for all patients. The studies show that outcomes are worse with increasing age and with cardiac or renal dysfunction at baseline. Renal excretion of sodium tends to worsen with increasing age and baseline renal dysfunction, as well as in those with preexisting cardiac or coronary disease, she explained.

Furthermore, patients with a history of hepatic encephalopathy tend to have worsening of the condition following TIPS.

“We also now know that in those with more severe liver disease, with shunting the liver becomes mildly ischemic, and by placing TIPS, we may actually precipitate liver failure,” Dr. Wong said.

TIPS can be considered in those under age 65 with normal cardiac and renal function, and without a history of hepatic encephalopathy or severe liver disease, she said.

DESTIN, FLA. — The incidence of gout is on the rise, and lifestyle factors are largely to blame, Dr. N. Lawrence Edwards said at the annual Rheumatology on the Beach.

For example, one study showed that between 1977–1978 and 1995–1996, the annual rate of primary gout more than doubled, from about 16 cases/100,000 population to nearly 42 cases/100,000 population. Factors that appear to play a role in this incidence spike are greater longevity, widespread diuretic and aspirin use, hypertension incidence, obesity, metabolic syndrome, and dietary trends, said Dr. Edwards, professor and vice chairman of the department of medicine at the University of Florida, Gainesville.

Certain dietary factors affect the risk for gout (high consumption of meat, seafood, and beer increase risk, and high intake of dairy is protective, for example); other risk factors are modifiable (such as obesity and hypertension). However, despite the association between lifestyle factors and risk, their modification will not eliminate existing gout. Weight reduction, decreased alcohol consumption, and reduced intake of purine-rich foods will reduce urate levels only by about 1 mg/dL. Medical treatment should be considered early in patients presenting with acute attacks, he said.

Urate-lowering therapy, which 15 years ago was reserved for use only in patients with chronic gout, now is considered warranted following the first one or two acute attacks, he noted.

Treatment goals for gout include termination of the attack as rapidly as possible, prevention of future attacks by reducing the chance of crystal-induced inflammation, and long-term correction of metabolic problems with a goal of lowering serum urate to below 6 mg/dL to allow depletion of the total body uric acid pool.

Uricosuric agents, such a probenecid, and the xanthine oxidase inhibitor allopurinol are used for this purpose.

These agents are used for reducing urate levels but uricosuric agents increase the risk of uric acid crystallization in the urine and associated stone formation. A number of other agents, such as ampicillin, penicillin, cephradine, heparin, and rifampicin, can potentially affect the action of uricosuric agents, Dr. Edwards said.

Allopurinol, which is a purine analog that is both a substrate and inhibitor of xanthine oxidase, is effective both for people who over produce and for those who underexcrete xanthine oxidase. The drug also has the convenience of single daily dosing, and it can be efficacious in patients with renal insufficiency.

However, allopurinol isn't always effective for achieving target serum urate levels. In one study of patients treated daily with a 300-mg dose, only about half achieved a serum urate level of 6 mg/dL, and in a large observational study of nearly 6,000 patients, median serum urate levels decreased from 8.7 mg/dL to 7.1 mg/dL. Although dosing up to 800 mg/day is acceptable, concerns about intolerance based on reports of severe hypersensitivity syndrome, rash, gastrointestinal problems, increases in liver enzymes, and rare bone marrow suppression tend to scare physicians away from prescribing higher doses, he noted. As with uricosuric agents, drug-drug interactions also are a problem with allopurinol.

Keys to effective treatment with this drug include dosing allopurinol to achieve a serum urate level between 5.0 and 6.0 mg/dL, which allows reduction of total body urate pool and mobilization of deposited crystals, Dr. Edwards said, stressing the need to start at a low dose of 50–100 mg/day, with close monitoring of escalation.

If the target serum urate level is reached, therapy should be ongoing; intermittent therapy or withdrawal will lead to recurrence of acute attacks within 6 months, and development of tophi within a few years.

Allopurinol shouldn't be started during an acute gouty attack and shouldn't be stopped in those already on treatment, he added. Also keep in mind that these medications will not control the pain of gout, and pain medications will do nothing to reduce serum urate, he advised.

New urate-lowering agents that improve compliance and are useful in those with allopurinol intolerance and renal failure, and who require treatment with other drugs that interact with allopurinol and uricosuric agents are needed, he concluded.

DESTIN, FLA. — The incidence of gout is on the rise, and lifestyle factors are largely to blame, Dr. N. Lawrence Edwards said at the annual Rheumatology on the Beach.

For example, one study showed that between 1977–1978 and 1995–1996, the annual rate of primary gout more than doubled, from about 16 cases/100,000 population to nearly 42 cases/100,000 population. Factors that appear to play a role in this incidence spike are greater longevity, widespread diuretic and aspirin use, hypertension incidence, obesity, metabolic syndrome, and dietary trends, said Dr. Edwards, professor and vice chairman of the department of medicine at the University of Florida, Gainesville.

Certain dietary factors affect the risk for gout (high consumption of meat, seafood, and beer increase risk, and high intake of dairy is protective, for example); other risk factors are modifiable (such as obesity and hypertension). However, despite the association between lifestyle factors and risk, their modification will not eliminate existing gout. Weight reduction, decreased alcohol consumption, and reduced intake of purine-rich foods will reduce urate levels only by about 1 mg/dL. Medical treatment should be considered early in patients presenting with acute attacks, he said.

Urate-lowering therapy, which 15 years ago was reserved for use only in patients with chronic gout, now is considered warranted following the first one or two acute attacks, he noted.

Treatment goals for gout include termination of the attack as rapidly as possible, prevention of future attacks by reducing the chance of crystal-induced inflammation, and long-term correction of metabolic problems with a goal of lowering serum urate to below 6 mg/dL to allow depletion of the total body uric acid pool.

Uricosuric agents, such a probenecid, and the xanthine oxidase inhibitor allopurinol are used for this purpose.

These agents are used for reducing urate levels but uricosuric agents increase the risk of uric acid crystallization in the urine and associated stone formation. A number of other agents, such as ampicillin, penicillin, cephradine, heparin, and rifampicin, can potentially affect the action of uricosuric agents, Dr. Edwards said.

Allopurinol, which is a purine analog that is both a substrate and inhibitor of xanthine oxidase, is effective both for people who over produce and for those who underexcrete xanthine oxidase. The drug also has the convenience of single daily dosing, and it can be efficacious in patients with renal insufficiency.

However, allopurinol isn't always effective for achieving target serum urate levels. In one study of patients treated daily with a 300-mg dose, only about half achieved a serum urate level of 6 mg/dL, and in a large observational study of nearly 6,000 patients, median serum urate levels decreased from 8.7 mg/dL to 7.1 mg/dL. Although dosing up to 800 mg/day is acceptable, concerns about intolerance based on reports of severe hypersensitivity syndrome, rash, gastrointestinal problems, increases in liver enzymes, and rare bone marrow suppression tend to scare physicians away from prescribing higher doses, he noted. As with uricosuric agents, drug-drug interactions also are a problem with allopurinol.

Keys to effective treatment with this drug include dosing allopurinol to achieve a serum urate level between 5.0 and 6.0 mg/dL, which allows reduction of total body urate pool and mobilization of deposited crystals, Dr. Edwards said, stressing the need to start at a low dose of 50–100 mg/day, with close monitoring of escalation.

If the target serum urate level is reached, therapy should be ongoing; intermittent therapy or withdrawal will lead to recurrence of acute attacks within 6 months, and development of tophi within a few years.

Allopurinol shouldn't be started during an acute gouty attack and shouldn't be stopped in those already on treatment, he added. Also keep in mind that these medications will not control the pain of gout, and pain medications will do nothing to reduce serum urate, he advised.

New urate-lowering agents that improve compliance and are useful in those with allopurinol intolerance and renal failure, and who require treatment with other drugs that interact with allopurinol and uricosuric agents are needed, he concluded.

DESTIN, FLA. — The incidence of gout is on the rise, and lifestyle factors are largely to blame, Dr. N. Lawrence Edwards said at the annual Rheumatology on the Beach.

For example, one study showed that between 1977–1978 and 1995–1996, the annual rate of primary gout more than doubled, from about 16 cases/100,000 population to nearly 42 cases/100,000 population. Factors that appear to play a role in this incidence spike are greater longevity, widespread diuretic and aspirin use, hypertension incidence, obesity, metabolic syndrome, and dietary trends, said Dr. Edwards, professor and vice chairman of the department of medicine at the University of Florida, Gainesville.

Certain dietary factors affect the risk for gout (high consumption of meat, seafood, and beer increase risk, and high intake of dairy is protective, for example); other risk factors are modifiable (such as obesity and hypertension). However, despite the association between lifestyle factors and risk, their modification will not eliminate existing gout. Weight reduction, decreased alcohol consumption, and reduced intake of purine-rich foods will reduce urate levels only by about 1 mg/dL. Medical treatment should be considered early in patients presenting with acute attacks, he said.

Urate-lowering therapy, which 15 years ago was reserved for use only in patients with chronic gout, now is considered warranted following the first one or two acute attacks, he noted.

Treatment goals for gout include termination of the attack as rapidly as possible, prevention of future attacks by reducing the chance of crystal-induced inflammation, and long-term correction of metabolic problems with a goal of lowering serum urate to below 6 mg/dL to allow depletion of the total body uric acid pool.

Uricosuric agents, such a probenecid, and the xanthine oxidase inhibitor allopurinol are used for this purpose.

These agents are used for reducing urate levels but uricosuric agents increase the risk of uric acid crystallization in the urine and associated stone formation. A number of other agents, such as ampicillin, penicillin, cephradine, heparin, and rifampicin, can potentially affect the action of uricosuric agents, Dr. Edwards said.

Allopurinol, which is a purine analog that is both a substrate and inhibitor of xanthine oxidase, is effective both for people who over produce and for those who underexcrete xanthine oxidase. The drug also has the convenience of single daily dosing, and it can be efficacious in patients with renal insufficiency.

However, allopurinol isn't always effective for achieving target serum urate levels. In one study of patients treated daily with a 300-mg dose, only about half achieved a serum urate level of 6 mg/dL, and in a large observational study of nearly 6,000 patients, median serum urate levels decreased from 8.7 mg/dL to 7.1 mg/dL. Although dosing up to 800 mg/day is acceptable, concerns about intolerance based on reports of severe hypersensitivity syndrome, rash, gastrointestinal problems, increases in liver enzymes, and rare bone marrow suppression tend to scare physicians away from prescribing higher doses, he noted. As with uricosuric agents, drug-drug interactions also are a problem with allopurinol.

Keys to effective treatment with this drug include dosing allopurinol to achieve a serum urate level between 5.0 and 6.0 mg/dL, which allows reduction of total body urate pool and mobilization of deposited crystals, Dr. Edwards said, stressing the need to start at a low dose of 50–100 mg/day, with close monitoring of escalation.

If the target serum urate level is reached, therapy should be ongoing; intermittent therapy or withdrawal will lead to recurrence of acute attacks within 6 months, and development of tophi within a few years.

Allopurinol shouldn't be started during an acute gouty attack and shouldn't be stopped in those already on treatment, he added. Also keep in mind that these medications will not control the pain of gout, and pain medications will do nothing to reduce serum urate, he advised.

New urate-lowering agents that improve compliance and are useful in those with allopurinol intolerance and renal failure, and who require treatment with other drugs that interact with allopurinol and uricosuric agents are needed, he concluded.

DESTIN, FLA. — With proper coding in place, billing for in-office infusion procedures can be as simple as billing for an office visit, Reuben A. Allen said at the annual Rheumatology on the Beach.

Infusion coding typically involves infusion procedure codes and drug codes for primary and secondary drugs, and can also include office visit codes, said Mr. Allen, a certified health care compliance consultant and certified management consultant who has a consulting practice in Wilmington, N.C.

Infusion coding is further broken down into complex, diagnostic, and hydration infusion procedures, with primary and secondary coding for each category. For example, with complex infusions, the primary code (96413) is used for the first hour. This code can be used for any complex infusion that exceeds 15 minutes. For each additional hour of infusion, physicians should use code 96415, keeping in mind that this code applies only to an infusion lasting at least 31 minutes following the initial or prior hour, thus it would only be used after 91 minutes of infusion, Mr. Allen noted. The first hour of diagnostic infusion procedures should be coded as 90765, and subsequent hours (the 31-minute rule applies here, as well) are coded as 90766. Hydration infusion codes (90760 for the first hour, and 90761 for subsequent hours) follow the same rules.

Code a first drug pushed as 90774, and each sequential drug pushed as 90775. The correct way to code methotrexate and diagnostic drugs is as subcutaneous or injection drugs. Only one primary code can be used per visit, he said. To code for office visits, there must be a reason for the visit. Billing for an office visit with every infusion will serve as a red flag. When warranted, it is appropriate to use Modifier 25 for the separate and distinct office visit service, keeping in mind that the diagnosis may be different for that than for the infusion.

DESTIN, FLA. — With proper coding in place, billing for in-office infusion procedures can be as simple as billing for an office visit, Reuben A. Allen said at the annual Rheumatology on the Beach.

Infusion coding typically involves infusion procedure codes and drug codes for primary and secondary drugs, and can also include office visit codes, said Mr. Allen, a certified health care compliance consultant and certified management consultant who has a consulting practice in Wilmington, N.C.

Infusion coding is further broken down into complex, diagnostic, and hydration infusion procedures, with primary and secondary coding for each category. For example, with complex infusions, the primary code (96413) is used for the first hour. This code can be used for any complex infusion that exceeds 15 minutes. For each additional hour of infusion, physicians should use code 96415, keeping in mind that this code applies only to an infusion lasting at least 31 minutes following the initial or prior hour, thus it would only be used after 91 minutes of infusion, Mr. Allen noted. The first hour of diagnostic infusion procedures should be coded as 90765, and subsequent hours (the 31-minute rule applies here, as well) are coded as 90766. Hydration infusion codes (90760 for the first hour, and 90761 for subsequent hours) follow the same rules.

Code a first drug pushed as 90774, and each sequential drug pushed as 90775. The correct way to code methotrexate and diagnostic drugs is as subcutaneous or injection drugs. Only one primary code can be used per visit, he said. To code for office visits, there must be a reason for the visit. Billing for an office visit with every infusion will serve as a red flag. When warranted, it is appropriate to use Modifier 25 for the separate and distinct office visit service, keeping in mind that the diagnosis may be different for that than for the infusion.

DESTIN, FLA. — With proper coding in place, billing for in-office infusion procedures can be as simple as billing for an office visit, Reuben A. Allen said at the annual Rheumatology on the Beach.

Infusion coding typically involves infusion procedure codes and drug codes for primary and secondary drugs, and can also include office visit codes, said Mr. Allen, a certified health care compliance consultant and certified management consultant who has a consulting practice in Wilmington, N.C.

Infusion coding is further broken down into complex, diagnostic, and hydration infusion procedures, with primary and secondary coding for each category. For example, with complex infusions, the primary code (96413) is used for the first hour. This code can be used for any complex infusion that exceeds 15 minutes. For each additional hour of infusion, physicians should use code 96415, keeping in mind that this code applies only to an infusion lasting at least 31 minutes following the initial or prior hour, thus it would only be used after 91 minutes of infusion, Mr. Allen noted. The first hour of diagnostic infusion procedures should be coded as 90765, and subsequent hours (the 31-minute rule applies here, as well) are coded as 90766. Hydration infusion codes (90760 for the first hour, and 90761 for subsequent hours) follow the same rules.

Code a first drug pushed as 90774, and each sequential drug pushed as 90775. The correct way to code methotrexate and diagnostic drugs is as subcutaneous or injection drugs. Only one primary code can be used per visit, he said. To code for office visits, there must be a reason for the visit. Billing for an office visit with every infusion will serve as a red flag. When warranted, it is appropriate to use Modifier 25 for the separate and distinct office visit service, keeping in mind that the diagnosis may be different for that than for the infusion.

DESTIN, FLA. — Treatment is not necessary in all patients with pulmonary sarcoidosis, Dr. Marc Judson said at the annual Rheumatology on the Beach.

More than two-thirds of patients with this form of sarcoidosis, which accounts for the vast majority of cases, will spontaneously remit. Thus the side effects of corticosteroids, the most common form of treatment, often are not worth the limited benefits, said Dr. Judson, professor of medicine at the Medical University of South Carolina, Charleston.

Furthermore, some retrospective evidence suggests corticosteroid treatment promotes relapse. In one study, more than 70% of treated patients relapsed, versus less than 10% who received no treatment, according to Dr. Judson. In another, the relapse rate was higher in patients who received a mean dose of 17 mg of prednisone daily, versus a mean dose of 10 mg daily.

He recommends using a decision analysis based on prognostic factors and degree of pulmonary function in patients with pulmonary sarcoidosis, whereby asymptomatic patients are untreated, and those with mild pulmonary dysfunction and minimal functional limitation are observed without treatment. These patients are likely to experience spontaneous remission.

Patients with an excellent prognosis (see sidebar) are also observed, but palliative care can be attempted when necessary—such as in those who develop severe sarcoid arthritis. Nonsteroidal anti-inflammatory drugs can help in these patients, he said.

In those with mild to moderate pulmonary dysfunction and mild to moderate functional limitation, treatment and observation for deterioration are both acceptable, but Dr. Judson recommends observation initially, with treatment for deterioration, and a steroid trial if no improvement is seen within 3–6 months.

Prednisone at 20–40 mg per day for 2–6 weeks is recommended in those who do undergo treatment. After the initial 2 to 6-week dosing regimen, the dose is tapered over 1–3 months to a maintenance dose, which is used for 3–9 months. The patient is then tapered off the drug over 1–3 months, followed by an observation period and a second trial in those who relapse.

Other treatment options include methotrexate, pentoxifylline, chloroquine, azathioprine, and infliximab. Methotrexate is the most studied and appears to have some benefit; azathioprine appears to have the least, he said.

Recent data on infliximab are promising. Dr. Judson and his colleagues found the overall change from baseline was 2.5% predicted forced vital capacity at 24 weeks in 93 patients treated with either 3 or 5 mg/kg of infliximab. The change was statistically significant. Furthermore, some patients had only mild disease; patients were also treated with prednisone; and the 2.5% change from baseline was in addition to benefits seen with prednisone.

Dr. Judson received research grants from Centocor, maker of infliximab.

ELSEVIER GLOBAL MEDICAL NEWS

Factors Can Predict Sarcoidosis Outcomes

Data suggest most patients with sarcoidosis will have spontaneous remission with or without treatment, while up to a third will develop chronic disease.

A literature review suggests there are several factors that can predict a good (likelihood of remission) or poor (likelihood of chronic disease development) prognosis.

Stage I vs. stage II-III disease as determined by chest x-ray and the presence of erythema nodosum appears to be linked to a good prognosis, Dr. Judson said.

The following predict poor prognosis: black race, extrathoracic disease, age 40 years or more, splenic involvement, lupus pernio, disease duration over 3 years, forced vital capacity less than 1.5 L, and stage IV disease/aspergilloma on chest x-ray. The latter two factors are also risk factors for death from sarcoidosis.

However, death from the disease occurs rarely (in 3%–5% of patients).

DESTIN, FLA. — Treatment is not necessary in all patients with pulmonary sarcoidosis, Dr. Marc Judson said at the annual Rheumatology on the Beach.

More than two-thirds of patients with this form of sarcoidosis, which accounts for the vast majority of cases, will spontaneously remit. Thus the side effects of corticosteroids, the most common form of treatment, often are not worth the limited benefits, said Dr. Judson, professor of medicine at the Medical University of South Carolina, Charleston.

Furthermore, some retrospective evidence suggests corticosteroid treatment promotes relapse. In one study, more than 70% of treated patients relapsed, versus less than 10% who received no treatment, according to Dr. Judson. In another, the relapse rate was higher in patients who received a mean dose of 17 mg of prednisone daily, versus a mean dose of 10 mg daily.

He recommends using a decision analysis based on prognostic factors and degree of pulmonary function in patients with pulmonary sarcoidosis, whereby asymptomatic patients are untreated, and those with mild pulmonary dysfunction and minimal functional limitation are observed without treatment. These patients are likely to experience spontaneous remission.

Patients with an excellent prognosis (see sidebar) are also observed, but palliative care can be attempted when necessary—such as in those who develop severe sarcoid arthritis. Nonsteroidal anti-inflammatory drugs can help in these patients, he said.

In those with mild to moderate pulmonary dysfunction and mild to moderate functional limitation, treatment and observation for deterioration are both acceptable, but Dr. Judson recommends observation initially, with treatment for deterioration, and a steroid trial if no improvement is seen within 3–6 months.

Prednisone at 20–40 mg per day for 2–6 weeks is recommended in those who do undergo treatment. After the initial 2 to 6-week dosing regimen, the dose is tapered over 1–3 months to a maintenance dose, which is used for 3–9 months. The patient is then tapered off the drug over 1–3 months, followed by an observation period and a second trial in those who relapse.

Other treatment options include methotrexate, pentoxifylline, chloroquine, azathioprine, and infliximab. Methotrexate is the most studied and appears to have some benefit; azathioprine appears to have the least, he said.

Recent data on infliximab are promising. Dr. Judson and his colleagues found the overall change from baseline was 2.5% predicted forced vital capacity at 24 weeks in 93 patients treated with either 3 or 5 mg/kg of infliximab. The change was statistically significant. Furthermore, some patients had only mild disease; patients were also treated with prednisone; and the 2.5% change from baseline was in addition to benefits seen with prednisone.

Dr. Judson received research grants from Centocor, maker of infliximab.

ELSEVIER GLOBAL MEDICAL NEWS

Factors Can Predict Sarcoidosis Outcomes

Data suggest most patients with sarcoidosis will have spontaneous remission with or without treatment, while up to a third will develop chronic disease.

A literature review suggests there are several factors that can predict a good (likelihood of remission) or poor (likelihood of chronic disease development) prognosis.

Stage I vs. stage II-III disease as determined by chest x-ray and the presence of erythema nodosum appears to be linked to a good prognosis, Dr. Judson said.

The following predict poor prognosis: black race, extrathoracic disease, age 40 years or more, splenic involvement, lupus pernio, disease duration over 3 years, forced vital capacity less than 1.5 L, and stage IV disease/aspergilloma on chest x-ray. The latter two factors are also risk factors for death from sarcoidosis.

However, death from the disease occurs rarely (in 3%–5% of patients).

DESTIN, FLA. — Treatment is not necessary in all patients with pulmonary sarcoidosis, Dr. Marc Judson said at the annual Rheumatology on the Beach.

More than two-thirds of patients with this form of sarcoidosis, which accounts for the vast majority of cases, will spontaneously remit. Thus the side effects of corticosteroids, the most common form of treatment, often are not worth the limited benefits, said Dr. Judson, professor of medicine at the Medical University of South Carolina, Charleston.

Furthermore, some retrospective evidence suggests corticosteroid treatment promotes relapse. In one study, more than 70% of treated patients relapsed, versus less than 10% who received no treatment, according to Dr. Judson. In another, the relapse rate was higher in patients who received a mean dose of 17 mg of prednisone daily, versus a mean dose of 10 mg daily.

He recommends using a decision analysis based on prognostic factors and degree of pulmonary function in patients with pulmonary sarcoidosis, whereby asymptomatic patients are untreated, and those with mild pulmonary dysfunction and minimal functional limitation are observed without treatment. These patients are likely to experience spontaneous remission.

Patients with an excellent prognosis (see sidebar) are also observed, but palliative care can be attempted when necessary—such as in those who develop severe sarcoid arthritis. Nonsteroidal anti-inflammatory drugs can help in these patients, he said.

In those with mild to moderate pulmonary dysfunction and mild to moderate functional limitation, treatment and observation for deterioration are both acceptable, but Dr. Judson recommends observation initially, with treatment for deterioration, and a steroid trial if no improvement is seen within 3–6 months.

Prednisone at 20–40 mg per day for 2–6 weeks is recommended in those who do undergo treatment. After the initial 2 to 6-week dosing regimen, the dose is tapered over 1–3 months to a maintenance dose, which is used for 3–9 months. The patient is then tapered off the drug over 1–3 months, followed by an observation period and a second trial in those who relapse.

Other treatment options include methotrexate, pentoxifylline, chloroquine, azathioprine, and infliximab. Methotrexate is the most studied and appears to have some benefit; azathioprine appears to have the least, he said.

Recent data on infliximab are promising. Dr. Judson and his colleagues found the overall change from baseline was 2.5% predicted forced vital capacity at 24 weeks in 93 patients treated with either 3 or 5 mg/kg of infliximab. The change was statistically significant. Furthermore, some patients had only mild disease; patients were also treated with prednisone; and the 2.5% change from baseline was in addition to benefits seen with prednisone.

Dr. Judson received research grants from Centocor, maker of infliximab.

ELSEVIER GLOBAL MEDICAL NEWS

Factors Can Predict Sarcoidosis Outcomes

Data suggest most patients with sarcoidosis will have spontaneous remission with or without treatment, while up to a third will develop chronic disease.

A literature review suggests there are several factors that can predict a good (likelihood of remission) or poor (likelihood of chronic disease development) prognosis.

Stage I vs. stage II-III disease as determined by chest x-ray and the presence of erythema nodosum appears to be linked to a good prognosis, Dr. Judson said.

The following predict poor prognosis: black race, extrathoracic disease, age 40 years or more, splenic involvement, lupus pernio, disease duration over 3 years, forced vital capacity less than 1.5 L, and stage IV disease/aspergilloma on chest x-ray. The latter two factors are also risk factors for death from sarcoidosis.

However, death from the disease occurs rarely (in 3%–5% of patients).