Pediatric, Adult Lupus Have Little in Common

CHICAGO — A substantial chasm exists between children and adults when it comes to the diagnosis, course, and treatment of lupus erythematosus, Dr. Kathleen M. O'Neil said at a symposium sponsored by the American College of Rheumatology.

“Kids with lupus are not adults with lupus,” she said, noting that children with prepubertal onset often do not have fatigue but they do have alopecia, whereas teens and adults are more likely to have fatigue. When asked, children will say they feel tired, but their fatigue levels still remain well below those of older kids, according to Dr. O'Neil, professor of pediatric rheumatology at the University of Oklahoma, Oklahoma City.

Children with vague and miscellaneous aches and pains often are evaluated for lupus, and there's a common misconception that a positive antinuclear antibody (ANA) test result is as reliable an indicator of lupus in children as it is in adults. While antinuclear antibody is positive in all children with systemic lupus erythematosus (SLE), an estimated “30%–35% of children who are healthy have a positive ANA, if we use the normal cutoff of 1:40 defined in adults,” she said in an interview.

In addition, the ANA does not discriminate healthy children from children with systemic lupus erythematosus (SLE) unless the titer used is 1:320 or greater. “A misdiagnosis puts parents through unnecessary stress and they get frightened, especially when they research lupus on the Internet,” she said.

Another lab finding that supports a diagnosis of SLE in children is positivity for anti-DNA antibodies, “which are present in 67% of our children with lupus onset over age 13 and 100% of those under age 13.” Evidence of complement consumption and low serum C3 and/or C4 concentrations are also supportive, Dr. O'Neil said.

Complement deficiency and early complement component deficiencies may account for 20% to 30% of prepubertal lupus onset, most easily identified with a CH50 screening test in all SLE children under the age of 10, she said. When a very young child develops SLE, environmental factors probably play less of a role than in the typical adult patient, suggesting a greater role for genetic factors.

In the absence of an approved drug regimen for children with lupus, treatment must be extrapolated from clinical experience and the results of clinical trials involving adult patients. “But we have to treat these children, because their disease is quite aggressive and they do die from renal failure,” said Dr. O'Neil.

The child's future fertility and bone and heart health can be adversely affected by such drugs as cyclophosphamide and steroids. Future fertility does concern adolescents, but they usually will not acknowledge that concern. “So it's important to do that for them.” Raise these issues with them, and realize that “if they say they're feeling fine, don't accept that. You still have to do a very careful and directed review of systems each time you see these young patients,” she said. “And when you're putting a 16- or 17-year-old boy on cyclophosphamide, don't forget to talk to him about sperm donation and storage.”

“Nonsteroidal anti-inflammatory drugs can be helpful in treating the arthritis, pericarditis, and pleuritis in lupus, but they have to be used with caution in case of underlying kidney disease,” she said. Exercise is important for maintaining bone density, especially in patients taking steroids.

Premature atherosclerosis can occur in the adolescent lupus patient, and treating procoagulant phenotype in children can be very tricky because the metabolism of young lupus patients is complex. Most pediatric hematologist-oncologists anticoagulate with low-molecular-weight heparin. “Children metabolize oral anticoagulants differently than adults do, and their diets are more varied, and that changes the absorption rate of the drug. Furthermore, they can get infections, which change drug metabolism, so their clotting times can vary widely,” Dr. O'Neil said.

Antiphospholipid antibodies in lupus increase coagulation, but the overall result of this can be variable. The pediatric SLE patient can be excessively anticoagulated one week and then be inadequately anticoagulated a week later. “By using low-molecular-weight heparin, it's easier to set the dosage, to predict what the drug is going to do, and to maintain a steady anticoagulation,” she said in an interview.

CHICAGO — A substantial chasm exists between children and adults when it comes to the diagnosis, course, and treatment of lupus erythematosus, Dr. Kathleen M. O'Neil said at a symposium sponsored by the American College of Rheumatology.

“Kids with lupus are not adults with lupus,” she said, noting that children with prepubertal onset often do not have fatigue but they do have alopecia, whereas teens and adults are more likely to have fatigue. When asked, children will say they feel tired, but their fatigue levels still remain well below those of older kids, according to Dr. O'Neil, professor of pediatric rheumatology at the University of Oklahoma, Oklahoma City.

Children with vague and miscellaneous aches and pains often are evaluated for lupus, and there's a common misconception that a positive antinuclear antibody (ANA) test result is as reliable an indicator of lupus in children as it is in adults. While antinuclear antibody is positive in all children with systemic lupus erythematosus (SLE), an estimated “30%–35% of children who are healthy have a positive ANA, if we use the normal cutoff of 1:40 defined in adults,” she said in an interview.

In addition, the ANA does not discriminate healthy children from children with systemic lupus erythematosus (SLE) unless the titer used is 1:320 or greater. “A misdiagnosis puts parents through unnecessary stress and they get frightened, especially when they research lupus on the Internet,” she said.

Another lab finding that supports a diagnosis of SLE in children is positivity for anti-DNA antibodies, “which are present in 67% of our children with lupus onset over age 13 and 100% of those under age 13.” Evidence of complement consumption and low serum C3 and/or C4 concentrations are also supportive, Dr. O'Neil said.

Complement deficiency and early complement component deficiencies may account for 20% to 30% of prepubertal lupus onset, most easily identified with a CH50 screening test in all SLE children under the age of 10, she said. When a very young child develops SLE, environmental factors probably play less of a role than in the typical adult patient, suggesting a greater role for genetic factors.

In the absence of an approved drug regimen for children with lupus, treatment must be extrapolated from clinical experience and the results of clinical trials involving adult patients. “But we have to treat these children, because their disease is quite aggressive and they do die from renal failure,” said Dr. O'Neil.

The child's future fertility and bone and heart health can be adversely affected by such drugs as cyclophosphamide and steroids. Future fertility does concern adolescents, but they usually will not acknowledge that concern. “So it's important to do that for them.” Raise these issues with them, and realize that “if they say they're feeling fine, don't accept that. You still have to do a very careful and directed review of systems each time you see these young patients,” she said. “And when you're putting a 16- or 17-year-old boy on cyclophosphamide, don't forget to talk to him about sperm donation and storage.”

“Nonsteroidal anti-inflammatory drugs can be helpful in treating the arthritis, pericarditis, and pleuritis in lupus, but they have to be used with caution in case of underlying kidney disease,” she said. Exercise is important for maintaining bone density, especially in patients taking steroids.

Premature atherosclerosis can occur in the adolescent lupus patient, and treating procoagulant phenotype in children can be very tricky because the metabolism of young lupus patients is complex. Most pediatric hematologist-oncologists anticoagulate with low-molecular-weight heparin. “Children metabolize oral anticoagulants differently than adults do, and their diets are more varied, and that changes the absorption rate of the drug. Furthermore, they can get infections, which change drug metabolism, so their clotting times can vary widely,” Dr. O'Neil said.

Antiphospholipid antibodies in lupus increase coagulation, but the overall result of this can be variable. The pediatric SLE patient can be excessively anticoagulated one week and then be inadequately anticoagulated a week later. “By using low-molecular-weight heparin, it's easier to set the dosage, to predict what the drug is going to do, and to maintain a steady anticoagulation,” she said in an interview.

CHICAGO — A substantial chasm exists between children and adults when it comes to the diagnosis, course, and treatment of lupus erythematosus, Dr. Kathleen M. O'Neil said at a symposium sponsored by the American College of Rheumatology.

“Kids with lupus are not adults with lupus,” she said, noting that children with prepubertal onset often do not have fatigue but they do have alopecia, whereas teens and adults are more likely to have fatigue. When asked, children will say they feel tired, but their fatigue levels still remain well below those of older kids, according to Dr. O'Neil, professor of pediatric rheumatology at the University of Oklahoma, Oklahoma City.

Children with vague and miscellaneous aches and pains often are evaluated for lupus, and there's a common misconception that a positive antinuclear antibody (ANA) test result is as reliable an indicator of lupus in children as it is in adults. While antinuclear antibody is positive in all children with systemic lupus erythematosus (SLE), an estimated “30%–35% of children who are healthy have a positive ANA, if we use the normal cutoff of 1:40 defined in adults,” she said in an interview.

In addition, the ANA does not discriminate healthy children from children with systemic lupus erythematosus (SLE) unless the titer used is 1:320 or greater. “A misdiagnosis puts parents through unnecessary stress and they get frightened, especially when they research lupus on the Internet,” she said.

Another lab finding that supports a diagnosis of SLE in children is positivity for anti-DNA antibodies, “which are present in 67% of our children with lupus onset over age 13 and 100% of those under age 13.” Evidence of complement consumption and low serum C3 and/or C4 concentrations are also supportive, Dr. O'Neil said.

Complement deficiency and early complement component deficiencies may account for 20% to 30% of prepubertal lupus onset, most easily identified with a CH50 screening test in all SLE children under the age of 10, she said. When a very young child develops SLE, environmental factors probably play less of a role than in the typical adult patient, suggesting a greater role for genetic factors.

In the absence of an approved drug regimen for children with lupus, treatment must be extrapolated from clinical experience and the results of clinical trials involving adult patients. “But we have to treat these children, because their disease is quite aggressive and they do die from renal failure,” said Dr. O'Neil.

The child's future fertility and bone and heart health can be adversely affected by such drugs as cyclophosphamide and steroids. Future fertility does concern adolescents, but they usually will not acknowledge that concern. “So it's important to do that for them.” Raise these issues with them, and realize that “if they say they're feeling fine, don't accept that. You still have to do a very careful and directed review of systems each time you see these young patients,” she said. “And when you're putting a 16- or 17-year-old boy on cyclophosphamide, don't forget to talk to him about sperm donation and storage.”

“Nonsteroidal anti-inflammatory drugs can be helpful in treating the arthritis, pericarditis, and pleuritis in lupus, but they have to be used with caution in case of underlying kidney disease,” she said. Exercise is important for maintaining bone density, especially in patients taking steroids.

Premature atherosclerosis can occur in the adolescent lupus patient, and treating procoagulant phenotype in children can be very tricky because the metabolism of young lupus patients is complex. Most pediatric hematologist-oncologists anticoagulate with low-molecular-weight heparin. “Children metabolize oral anticoagulants differently than adults do, and their diets are more varied, and that changes the absorption rate of the drug. Furthermore, they can get infections, which change drug metabolism, so their clotting times can vary widely,” Dr. O'Neil said.

Antiphospholipid antibodies in lupus increase coagulation, but the overall result of this can be variable. The pediatric SLE patient can be excessively anticoagulated one week and then be inadequately anticoagulated a week later. “By using low-molecular-weight heparin, it's easier to set the dosage, to predict what the drug is going to do, and to maintain a steady anticoagulation,” she said in an interview.