• The percentage of the hospital’s total patient volume (for the population the group serves, i.e., adult versus children versus both) that the hospital medicine group (HMG) was responsible for caring for
• The presence of medical hospitalists within the HMG focusing their practice on a specific medical subspecialty, such as critical care, neurology, or oncology
• The value of CME allowances for hospitalists
• The utilization of prolonged service codes by hospitalists
• Charge capture methodologies being used by HMGs
• For academic HMGs, the dollar amount of financial support provided for non-clinical work

Order your copy now and improve your practice today at www.hospitalmedicine.org/survey.

• The percentage of the hospital’s total patient volume (for the population the group serves, i.e., adult versus children versus both) that the hospital medicine group (HMG) was responsible for caring for
• The presence of medical hospitalists within the HMG focusing their practice on a specific medical subspecialty, such as critical care, neurology, or oncology
• The value of CME allowances for hospitalists
• The utilization of prolonged service codes by hospitalists
• Charge capture methodologies being used by HMGs
• For academic HMGs, the dollar amount of financial support provided for non-clinical work

Order your copy now and improve your practice today at www.hospitalmedicine.org/survey.

• The percentage of the hospital’s total patient volume (for the population the group serves, i.e., adult versus children versus both) that the hospital medicine group (HMG) was responsible for caring for
• The presence of medical hospitalists within the HMG focusing their practice on a specific medical subspecialty, such as critical care, neurology, or oncology
• The value of CME allowances for hospitalists
• The utilization of prolonged service codes by hospitalists
• Charge capture methodologies being used by HMGs
• For academic HMGs, the dollar amount of financial support provided for non-clinical work

Order your copy now and improve your practice today at www.hospitalmedicine.org/survey.

Antihemophilic factor

Japan’s Ministry of Health, Labour and Welfare (MHLW) has approved albutrepenonacog alfa (Idelvion) to treat and prevent bleeding in children and adults with hemophilia B.

Albutrepenonacog alfa is a fusion protein linking recombinant coagulation factor IX with recombinant albumin.

The product is now approved for use as routine prophylaxis, for on-demand control of bleeding, and for perioperative management of bleeding.

Albutrepenonacog alfa is being developed by CSL Behring.

The company says the product is the first and only hemophilia B therapy with up to 14-day dosing intervals.

According to CSL Behring, albutrepenonacog alfa can deliver high-level protection from bleeds by maintaining factor IX activity levels at an average of 20% in patients treated prophylactically every 7 days and an average of 12% in patients treated prophylactically every 14 days.

Albutrepenonacog alfa has also been approved in Canada, the European Union, Switzerland, and the US.

Phase 3 trial

The MHLW approved albutrepenonacog alfa based on results of the PROLONG-9FP clinical development program. PROLONG-9FP includes phase 1, 2, and 3 studies evaluating the safety and efficacy of albutrepenonacog alfa in adults and children (ages 1 to 61) with hemophilia B.

Data from the phase 3 study were published in Blood. The study included 63 previously treated male patients with severe hemophilia B. They had a mean age of 33 (range, 12 to 61).

The patients were divided into 2 groups. Group 1 (n=40) received routine prophylaxis with albutrepenonacog alfa once every 7 days for 26 weeks, followed by a 7-, 10-, or 14-day prophylaxis regimen for a mean of 50, 38, or 51 weeks, respectively.

Group 2 received on-demand treatment with albutrepenonacog alfa for bleeding episodes for 26 weeks (n=23) and then switched to a 7-day prophylaxis regimen for a mean of 45 weeks (n=19).

The median annualized bleeding rate (ABR) was 2.0 in the prophylaxis arm (group 1) and 23.5 in the on-demand treatment arm (group 2). The median spontaneous ABRs were 0.0 and 17.0, respectively.

For patients in group 2, there was a significant reduction in median ABRs when patients switched from on-demand treatment to prophylaxis—19.22 and 1.58, respectively (P<0.0001). And there was a significant reduction in median spontaneous ABRs—15.43 and 0.00, respectively (P<0.0001).

Overall, 98.6% of bleeding episodes were treated successfully, including 93.6% that were treated with a single injection of albutrepenonacog alfa.

None of the patients developed inhibitors or experienced thromboembolic events, anaphylaxis, or life-threatening adverse events (AEs).

There were 347 treatment-emergent AEs reported in 54 (85.7%) patients. The most common were nasopharyngitis (25.4%), headache (23.8%), arthralgia (4.3%), and influenza (11.1%).

Eleven mild/moderate AEs in 5 patients (7.9%) were considered possibly related to albutrepenonacog alfa. Two patients discontinued treatment due to AEs—1 with hypersensitivity and 1 with headache.

Antihemophilic factor

Japan’s Ministry of Health, Labour and Welfare (MHLW) has approved albutrepenonacog alfa (Idelvion) to treat and prevent bleeding in children and adults with hemophilia B.

Albutrepenonacog alfa is a fusion protein linking recombinant coagulation factor IX with recombinant albumin.

The product is now approved for use as routine prophylaxis, for on-demand control of bleeding, and for perioperative management of bleeding.

Albutrepenonacog alfa is being developed by CSL Behring.

The company says the product is the first and only hemophilia B therapy with up to 14-day dosing intervals.

According to CSL Behring, albutrepenonacog alfa can deliver high-level protection from bleeds by maintaining factor IX activity levels at an average of 20% in patients treated prophylactically every 7 days and an average of 12% in patients treated prophylactically every 14 days.

Albutrepenonacog alfa has also been approved in Canada, the European Union, Switzerland, and the US.

Phase 3 trial

The MHLW approved albutrepenonacog alfa based on results of the PROLONG-9FP clinical development program. PROLONG-9FP includes phase 1, 2, and 3 studies evaluating the safety and efficacy of albutrepenonacog alfa in adults and children (ages 1 to 61) with hemophilia B.

Data from the phase 3 study were published in Blood. The study included 63 previously treated male patients with severe hemophilia B. They had a mean age of 33 (range, 12 to 61).

The patients were divided into 2 groups. Group 1 (n=40) received routine prophylaxis with albutrepenonacog alfa once every 7 days for 26 weeks, followed by a 7-, 10-, or 14-day prophylaxis regimen for a mean of 50, 38, or 51 weeks, respectively.

Group 2 received on-demand treatment with albutrepenonacog alfa for bleeding episodes for 26 weeks (n=23) and then switched to a 7-day prophylaxis regimen for a mean of 45 weeks (n=19).

The median annualized bleeding rate (ABR) was 2.0 in the prophylaxis arm (group 1) and 23.5 in the on-demand treatment arm (group 2). The median spontaneous ABRs were 0.0 and 17.0, respectively.

For patients in group 2, there was a significant reduction in median ABRs when patients switched from on-demand treatment to prophylaxis—19.22 and 1.58, respectively (P<0.0001). And there was a significant reduction in median spontaneous ABRs—15.43 and 0.00, respectively (P<0.0001).

Overall, 98.6% of bleeding episodes were treated successfully, including 93.6% that were treated with a single injection of albutrepenonacog alfa.

None of the patients developed inhibitors or experienced thromboembolic events, anaphylaxis, or life-threatening adverse events (AEs).

There were 347 treatment-emergent AEs reported in 54 (85.7%) patients. The most common were nasopharyngitis (25.4%), headache (23.8%), arthralgia (4.3%), and influenza (11.1%).

Eleven mild/moderate AEs in 5 patients (7.9%) were considered possibly related to albutrepenonacog alfa. Two patients discontinued treatment due to AEs—1 with hypersensitivity and 1 with headache.

Antihemophilic factor

Japan’s Ministry of Health, Labour and Welfare (MHLW) has approved albutrepenonacog alfa (Idelvion) to treat and prevent bleeding in children and adults with hemophilia B.

Albutrepenonacog alfa is a fusion protein linking recombinant coagulation factor IX with recombinant albumin.

The product is now approved for use as routine prophylaxis, for on-demand control of bleeding, and for perioperative management of bleeding.

Albutrepenonacog alfa is being developed by CSL Behring.

The company says the product is the first and only hemophilia B therapy with up to 14-day dosing intervals.

According to CSL Behring, albutrepenonacog alfa can deliver high-level protection from bleeds by maintaining factor IX activity levels at an average of 20% in patients treated prophylactically every 7 days and an average of 12% in patients treated prophylactically every 14 days.

Albutrepenonacog alfa has also been approved in Canada, the European Union, Switzerland, and the US.

Phase 3 trial

The MHLW approved albutrepenonacog alfa based on results of the PROLONG-9FP clinical development program. PROLONG-9FP includes phase 1, 2, and 3 studies evaluating the safety and efficacy of albutrepenonacog alfa in adults and children (ages 1 to 61) with hemophilia B.

Data from the phase 3 study were published in Blood. The study included 63 previously treated male patients with severe hemophilia B. They had a mean age of 33 (range, 12 to 61).

The patients were divided into 2 groups. Group 1 (n=40) received routine prophylaxis with albutrepenonacog alfa once every 7 days for 26 weeks, followed by a 7-, 10-, or 14-day prophylaxis regimen for a mean of 50, 38, or 51 weeks, respectively.

Group 2 received on-demand treatment with albutrepenonacog alfa for bleeding episodes for 26 weeks (n=23) and then switched to a 7-day prophylaxis regimen for a mean of 45 weeks (n=19).

The median annualized bleeding rate (ABR) was 2.0 in the prophylaxis arm (group 1) and 23.5 in the on-demand treatment arm (group 2). The median spontaneous ABRs were 0.0 and 17.0, respectively.

For patients in group 2, there was a significant reduction in median ABRs when patients switched from on-demand treatment to prophylaxis—19.22 and 1.58, respectively (P<0.0001). And there was a significant reduction in median spontaneous ABRs—15.43 and 0.00, respectively (P<0.0001).

Overall, 98.6% of bleeding episodes were treated successfully, including 93.6% that were treated with a single injection of albutrepenonacog alfa.

None of the patients developed inhibitors or experienced thromboembolic events, anaphylaxis, or life-threatening adverse events (AEs).

There were 347 treatment-emergent AEs reported in 54 (85.7%) patients. The most common were nasopharyngitis (25.4%), headache (23.8%), arthralgia (4.3%), and influenza (11.1%).

Eleven mild/moderate AEs in 5 patients (7.9%) were considered possibly related to albutrepenonacog alfa. Two patients discontinued treatment due to AEs—1 with hypersensitivity and 1 with headache.

A 1-week course of antibiotics is sufficient for most hospital-acquired and ventilator-associated pneumonia, regardless of the microbial etiology of the infection, according to an updated Clinical Practice Guidelines for managing adults with these disorders.

In addition, every hospital should develop its own antibiogram to align clinicians’ choice of treatments with the local distribution of likely pathogens and their antimicrobial susceptibilities. Both of these recommendations, as well as others that are also new to the updated guidelines, are intended to minimize patient exposure to unnecessary antibiotics and reduce antibiotic resistance, said Andre C. Kalil, MD, and Mark L. Metersky, MD, cochairs of the guidelines panel of 18 experts in infectious diseases, pulmonary medicine, critical care medicine, laboratory medicine, microbiology, pharmacology, and guideline methodology.

A 1-week course of antibiotics is sufficient for most hospital-acquired and ventilator-associated pneumonia, regardless of the microbial etiology of the infection, according to an updated Clinical Practice Guidelines for managing adults with these disorders.

In addition, every hospital should develop its own antibiogram to align clinicians’ choice of treatments with the local distribution of likely pathogens and their antimicrobial susceptibilities. Both of these recommendations, as well as others that are also new to the updated guidelines, are intended to minimize patient exposure to unnecessary antibiotics and reduce antibiotic resistance, said Andre C. Kalil, MD, and Mark L. Metersky, MD, cochairs of the guidelines panel of 18 experts in infectious diseases, pulmonary medicine, critical care medicine, laboratory medicine, microbiology, pharmacology, and guideline methodology.

A 1-week course of antibiotics is sufficient for most hospital-acquired and ventilator-associated pneumonia, regardless of the microbial etiology of the infection, according to an updated Clinical Practice Guidelines for managing adults with these disorders.

In addition, every hospital should develop its own antibiogram to align clinicians’ choice of treatments with the local distribution of likely pathogens and their antimicrobial susceptibilities. Both of these recommendations, as well as others that are also new to the updated guidelines, are intended to minimize patient exposure to unnecessary antibiotics and reduce antibiotic resistance, said Andre C. Kalil, MD, and Mark L. Metersky, MD, cochairs of the guidelines panel of 18 experts in infectious diseases, pulmonary medicine, critical care medicine, laboratory medicine, microbiology, pharmacology, and guideline methodology.

A 30-year-old man presented with multiple asymptomatic skin-colored and yellowish papules and nodules over the elbows, wrists, feet, buttocks, hands, and forearms (Figure 1) that had appeared suddenly 2 weeks before.

His medical history included diabetes mellitus, Hansen disease diagnosed 6 years earlier and treated with a multibacillary regimen for 1 year, and pancreatitis diagnosed 6 months earlier.

Laboratory testing. When a serum sample was centrifuged at 1,500 rpm for 15 minutes, a large lipid layer formed at the top (Figure 2). Other results:

• Triglycerides 5,742 mg/dL (reference range < 160)
• Total cholesterol 293 mg/dL (< 240 mg/dL)
• Fasting blood glucose 473 mg/dL
• Low-density and high-density lipoprotein cholesterol levels normal
• Complete blood cell count within normal limits.

Other testing. The electrocardiogram was normal. Retinal examination showed evidence of lipemia retinalis. Histologic study of a biopsy specimen from one of the lesions showed foamy macrophages in the superficial dermis with lymphocytic infiltrate, confirming the diagnosis of eruptive xanthoma.

An urgent medical referral was sought. The patient was started on statins and fibrates, and the treatment resulted in remarkable improvement.

ERUPTIVE XANTHOMA

Cutaneous manifestations can be warning signs of systemic disease, and physicians should be aware of the dermatologic presentations of common medical conditions.

Eruptive xanthoma is a sign of severe hypertriglyceridemia and is almost always due to an acquired cause such as hyperchylomicro­nemia or an inherited lipoprotein disorder (eg, lipoprotein lipase deficiency in children, common familial hypertriglyceridemia type 5 in adults).¹

Chylomicronemia syndrome is characterized by triglyceride levels greater than 1,000 mg/dL in a patient with eruptive xanthoma, lipemia retinalis, or abdominal pain or pancreatitis. The syndrome has a prevalence of 1.7 out of 10,000 patients.² Treatment is a strict low-fat diet, with a minimal role for fibrates and nicotinic acid.

Eruptive xanthoma is seen at the time of presentation in 8.5% of patients with severe hypertriglyceridemia (serum level > 1,772 mg/dL).³ In patients with serum triglyceride levels greater than 1,000 mg/dL, the risk of acute pancreatitis is 5%, and at levels above 2,000 mg/dL, the risk is 10% to 20%.⁴

In our patient, the diagnosis of chylomicronemia was based on the appearance of the skin lesions, his history of pancreatitis and diabetes mellitus, and the results of the initial workup, which revealed severe hypertriglyceridemia and lipemia retinalis. The presence of eruptive xanthomas should raise suspicion for a grossly elevated triglyceride level. Therefore, in view of the increased risk of acute pancreatitis and pancreatic necrosis associated with chylomicronemic syndrome,² recognizing the cutaneous signs is mandatory.

A 30-year-old man presented with multiple asymptomatic skin-colored and yellowish papules and nodules over the elbows, wrists, feet, buttocks, hands, and forearms (Figure 1) that had appeared suddenly 2 weeks before.

His medical history included diabetes mellitus, Hansen disease diagnosed 6 years earlier and treated with a multibacillary regimen for 1 year, and pancreatitis diagnosed 6 months earlier.

Laboratory testing. When a serum sample was centrifuged at 1,500 rpm for 15 minutes, a large lipid layer formed at the top (Figure 2). Other results:

• Triglycerides 5,742 mg/dL (reference range < 160)
• Total cholesterol 293 mg/dL (< 240 mg/dL)
• Fasting blood glucose 473 mg/dL
• Low-density and high-density lipoprotein cholesterol levels normal
• Complete blood cell count within normal limits.

Other testing. The electrocardiogram was normal. Retinal examination showed evidence of lipemia retinalis. Histologic study of a biopsy specimen from one of the lesions showed foamy macrophages in the superficial dermis with lymphocytic infiltrate, confirming the diagnosis of eruptive xanthoma.

An urgent medical referral was sought. The patient was started on statins and fibrates, and the treatment resulted in remarkable improvement.

ERUPTIVE XANTHOMA

Cutaneous manifestations can be warning signs of systemic disease, and physicians should be aware of the dermatologic presentations of common medical conditions.

Eruptive xanthoma is a sign of severe hypertriglyceridemia and is almost always due to an acquired cause such as hyperchylomicro­nemia or an inherited lipoprotein disorder (eg, lipoprotein lipase deficiency in children, common familial hypertriglyceridemia type 5 in adults).¹

Chylomicronemia syndrome is characterized by triglyceride levels greater than 1,000 mg/dL in a patient with eruptive xanthoma, lipemia retinalis, or abdominal pain or pancreatitis. The syndrome has a prevalence of 1.7 out of 10,000 patients.² Treatment is a strict low-fat diet, with a minimal role for fibrates and nicotinic acid.

Eruptive xanthoma is seen at the time of presentation in 8.5% of patients with severe hypertriglyceridemia (serum level > 1,772 mg/dL).³ In patients with serum triglyceride levels greater than 1,000 mg/dL, the risk of acute pancreatitis is 5%, and at levels above 2,000 mg/dL, the risk is 10% to 20%.⁴

In our patient, the diagnosis of chylomicronemia was based on the appearance of the skin lesions, his history of pancreatitis and diabetes mellitus, and the results of the initial workup, which revealed severe hypertriglyceridemia and lipemia retinalis. The presence of eruptive xanthomas should raise suspicion for a grossly elevated triglyceride level. Therefore, in view of the increased risk of acute pancreatitis and pancreatic necrosis associated with chylomicronemic syndrome,² recognizing the cutaneous signs is mandatory.

A 30-year-old man presented with multiple asymptomatic skin-colored and yellowish papules and nodules over the elbows, wrists, feet, buttocks, hands, and forearms (Figure 1) that had appeared suddenly 2 weeks before.

His medical history included diabetes mellitus, Hansen disease diagnosed 6 years earlier and treated with a multibacillary regimen for 1 year, and pancreatitis diagnosed 6 months earlier.

Laboratory testing. When a serum sample was centrifuged at 1,500 rpm for 15 minutes, a large lipid layer formed at the top (Figure 2). Other results:

• Triglycerides 5,742 mg/dL (reference range < 160)
• Total cholesterol 293 mg/dL (< 240 mg/dL)
• Fasting blood glucose 473 mg/dL
• Low-density and high-density lipoprotein cholesterol levels normal
• Complete blood cell count within normal limits.

Other testing. The electrocardiogram was normal. Retinal examination showed evidence of lipemia retinalis. Histologic study of a biopsy specimen from one of the lesions showed foamy macrophages in the superficial dermis with lymphocytic infiltrate, confirming the diagnosis of eruptive xanthoma.

An urgent medical referral was sought. The patient was started on statins and fibrates, and the treatment resulted in remarkable improvement.

ERUPTIVE XANTHOMA

Cutaneous manifestations can be warning signs of systemic disease, and physicians should be aware of the dermatologic presentations of common medical conditions.

Eruptive xanthoma is a sign of severe hypertriglyceridemia and is almost always due to an acquired cause such as hyperchylomicro­nemia or an inherited lipoprotein disorder (eg, lipoprotein lipase deficiency in children, common familial hypertriglyceridemia type 5 in adults).¹

Chylomicronemia syndrome is characterized by triglyceride levels greater than 1,000 mg/dL in a patient with eruptive xanthoma, lipemia retinalis, or abdominal pain or pancreatitis. The syndrome has a prevalence of 1.7 out of 10,000 patients.² Treatment is a strict low-fat diet, with a minimal role for fibrates and nicotinic acid.

Eruptive xanthoma is seen at the time of presentation in 8.5% of patients with severe hypertriglyceridemia (serum level > 1,772 mg/dL).³ In patients with serum triglyceride levels greater than 1,000 mg/dL, the risk of acute pancreatitis is 5%, and at levels above 2,000 mg/dL, the risk is 10% to 20%.⁴

In our patient, the diagnosis of chylomicronemia was based on the appearance of the skin lesions, his history of pancreatitis and diabetes mellitus, and the results of the initial workup, which revealed severe hypertriglyceridemia and lipemia retinalis. The presence of eruptive xanthomas should raise suspicion for a grossly elevated triglyceride level. Therefore, in view of the increased risk of acute pancreatitis and pancreatic necrosis associated with chylomicronemic syndrome,² recognizing the cutaneous signs is mandatory.

A 66-year-old woman presented with fever, cough, odynophagia, and anterior neck pain.

Examination of the oral cavity showed a swollen, erythematous uvula with exudate consistent with uvulitis (Figure 1). A lateral radiograph of the neck showed minimal thickening of the epiglottis (Figure 2). Fiberoptic laryngoscopy showed ulcerations along the base of the tongue, epiglottis, and aryepiglottic folds.

Intravenous antibiotics (ceftriaxone and vancomycin), intravenous corticosteroids, and acyclovir were started empirically, and the patient was admitted to the intensive care unit for observation of her respiratory status.

Results of rapid testing for group A streptococci were negative. Routine throat cultures were positive for group G beta-hemolytic streptococci. Viral throat cultures were negative for herpes simplex virus and cytomegalovirus. Multiplex polymerase chain reaction testing of a nasopharyngeal specimen was negative for a variety of respiratory viral pathogens. No organisms were identified on blood cultures.

Her fever and symptoms resolved. She was discharged home on an oral course of amoxicillin-clavulanic acid and acyclovir. Acyclovir was given based on the ulcerative lesions suggestive of herpetic infection. No recurrence was reported.

KEY FEATURES AND MANAGEMENT

Uvulitis is an uncommon infection, usually caused by Streptococcus pyogenes, S pneumoniae, or Haemophilus influenzae.^1–3 It can be an isolated finding or associated with concurrent pharyngitis and epiglottitis.^1–3

Uvulitis and epiglottitis can have similar presenting symptoms such as fever, sore throat, and odynophagia. Lateral neck radiography looking for an enlarged epiglottis (“thumb” sign) is recommended, given the similarities in presentation to uvulitis and the seriousness of epiglottitis if missed. If there are signs of airway obstruction, laryngoscopy should be performed in a controlled setting such as the intensive care unit, as it may precipitate sudden airway obstruction.

Close observation in the intensive care unit is recommended in adults presenting with epiglottitis because of the risk of rapid deterioration and the need to secure the airway. Empirical therapy with intravenous antibiotics (eg, a third-generation cephalosporin or a beta-lactamase inhibitor combination) to cover the common pathogens mentioned above is recommended and should then be tailored according to the results of blood culture testing.

A 66-year-old woman presented with fever, cough, odynophagia, and anterior neck pain.

Examination of the oral cavity showed a swollen, erythematous uvula with exudate consistent with uvulitis (Figure 1). A lateral radiograph of the neck showed minimal thickening of the epiglottis (Figure 2). Fiberoptic laryngoscopy showed ulcerations along the base of the tongue, epiglottis, and aryepiglottic folds.

Intravenous antibiotics (ceftriaxone and vancomycin), intravenous corticosteroids, and acyclovir were started empirically, and the patient was admitted to the intensive care unit for observation of her respiratory status.

Results of rapid testing for group A streptococci were negative. Routine throat cultures were positive for group G beta-hemolytic streptococci. Viral throat cultures were negative for herpes simplex virus and cytomegalovirus. Multiplex polymerase chain reaction testing of a nasopharyngeal specimen was negative for a variety of respiratory viral pathogens. No organisms were identified on blood cultures.

Her fever and symptoms resolved. She was discharged home on an oral course of amoxicillin-clavulanic acid and acyclovir. Acyclovir was given based on the ulcerative lesions suggestive of herpetic infection. No recurrence was reported.

KEY FEATURES AND MANAGEMENT

Uvulitis is an uncommon infection, usually caused by Streptococcus pyogenes, S pneumoniae, or Haemophilus influenzae.^1–3 It can be an isolated finding or associated with concurrent pharyngitis and epiglottitis.^1–3

Uvulitis and epiglottitis can have similar presenting symptoms such as fever, sore throat, and odynophagia. Lateral neck radiography looking for an enlarged epiglottis (“thumb” sign) is recommended, given the similarities in presentation to uvulitis and the seriousness of epiglottitis if missed. If there are signs of airway obstruction, laryngoscopy should be performed in a controlled setting such as the intensive care unit, as it may precipitate sudden airway obstruction.

Close observation in the intensive care unit is recommended in adults presenting with epiglottitis because of the risk of rapid deterioration and the need to secure the airway. Empirical therapy with intravenous antibiotics (eg, a third-generation cephalosporin or a beta-lactamase inhibitor combination) to cover the common pathogens mentioned above is recommended and should then be tailored according to the results of blood culture testing.

A 66-year-old woman presented with fever, cough, odynophagia, and anterior neck pain.

Examination of the oral cavity showed a swollen, erythematous uvula with exudate consistent with uvulitis (Figure 1). A lateral radiograph of the neck showed minimal thickening of the epiglottis (Figure 2). Fiberoptic laryngoscopy showed ulcerations along the base of the tongue, epiglottis, and aryepiglottic folds.

Intravenous antibiotics (ceftriaxone and vancomycin), intravenous corticosteroids, and acyclovir were started empirically, and the patient was admitted to the intensive care unit for observation of her respiratory status.

Results of rapid testing for group A streptococci were negative. Routine throat cultures were positive for group G beta-hemolytic streptococci. Viral throat cultures were negative for herpes simplex virus and cytomegalovirus. Multiplex polymerase chain reaction testing of a nasopharyngeal specimen was negative for a variety of respiratory viral pathogens. No organisms were identified on blood cultures.

Her fever and symptoms resolved. She was discharged home on an oral course of amoxicillin-clavulanic acid and acyclovir. Acyclovir was given based on the ulcerative lesions suggestive of herpetic infection. No recurrence was reported.

KEY FEATURES AND MANAGEMENT

Uvulitis is an uncommon infection, usually caused by Streptococcus pyogenes, S pneumoniae, or Haemophilus influenzae.^1–3 It can be an isolated finding or associated with concurrent pharyngitis and epiglottitis.^1–3

Uvulitis and epiglottitis can have similar presenting symptoms such as fever, sore throat, and odynophagia. Lateral neck radiography looking for an enlarged epiglottis (“thumb” sign) is recommended, given the similarities in presentation to uvulitis and the seriousness of epiglottitis if missed. If there are signs of airway obstruction, laryngoscopy should be performed in a controlled setting such as the intensive care unit, as it may precipitate sudden airway obstruction.

Close observation in the intensive care unit is recommended in adults presenting with epiglottitis because of the risk of rapid deterioration and the need to secure the airway. Empirical therapy with intravenous antibiotics (eg, a third-generation cephalosporin or a beta-lactamase inhibitor combination) to cover the common pathogens mentioned above is recommended and should then be tailored according to the results of blood culture testing.

A 68-year-old man with metastatic periampullary adenocarcinoma presented to his usual clinic for a scheduled biliary stent exchange by endoscopic retrograde cholangiopancreatography (ERCP). The stent had been placed 5 months before, and no complications had been reported during that procedure.

During the stent exchange procedure, the endoscopist advanced the scope to the second part of the duodenum, where a large, ulcerated, friable mass was visualized surrounding the ampulla, consistent with patient’s known periampullary cancer. The biliary stent was removed without much difficulty. However, several attempts to cannulate the common bile duct with a preloaded guidewire failed because of extensive edema and tissue friability, and to avoid further discomfort to the patient, the procedure was aborted. No perforation was visualized during or at the end of the procedure.

During the first hour after the procedure was stopped, the patient suddenly developed abdominal pain and distention and crepitus of the right chest wall. Supine abdominal radiography showed extensive pneumoperitoneum and subcutaneous emphysema in the chest. A nasogastric tube was placed for decompression, and the patient was transferred to the surgical intensive care unit at our hospital.

EVIDENCE OF PERFORATION NOTED

On arrival, the patient’s oxygen saturation was 99% while receiving oxygen at 2 L/minute by nasal cannula. The physical examination revealed neck swelling, abdominal distention, and crepitus in the neck, abdomen, scrotum, and right lower extremity.

Computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast revealed widespread pneumoretroperitoneum, pneumoperitoneum, and air along the intermuscular planes in the right lower extremity, with no evidence of extravasation of oral contrast (Figure 1). Also noted were bilateral pneumothorax, pneumomediastinum, pneumopericardium, and extensive subcutaneous emphysema (Figure 2).

Despite these impressive findings, the patient remained hemodynamically stable and was managed conservatively with broad-spectrum antibiotics, gastric decompression, and bowel rest. But repeat chest radiography 5 hours after admission to the hospital revealed an enlarging right pneumothorax, which was treated with placement of a pigtail catheter. The patient continued to improve with conservative management and was discharged on the 6th day of hospitalization.

PERFORATION DURING ERCP: INCIDENCE AND COMPLICATIONS

Although perforation is an uncommon complication of ERCP, with an incidence of 1%, mortality rates as high as 18% have been reported.¹ Older age, longer procedural time, anatomic variations, and diseases of the duodenum and common bile duct can increase the risk of perforation.²

Types of perforation

Stapfer et al¹ classified perforation during ERCP into four types, based on etiology and  site of perforation. Type 1 is perforation of the lateral or medial duodenal wall caused by excessive pressure from the endoscope or its acute angulation. Type 2 is periampullary injury, often associated with sphincterotomy or difficulty accessing the biliary tree. Type 3 is injury to the common bile duct or pancreatic duct caused by instrumentation. Type 4 is the presence of retroperitoneal free air with no evidence of actual perforation; this is usually an incidental finding and is of little or no clinical consequence.¹

In 2015, a review of 18 studies described the distribution of ERCP perforation according to the Stapfer classification: 25% were type 1, 46% were type 2, and 22% were type 3.³

Effects of air insufflation

ERCP requires air insufflation for optimal visualization. During difficult or prolonged procedures, a larger amount of air may be insufflated to maintain bowel lumen visibility. Depending on the site and size of the defect, a variable amount of air can leak under pressure once the perforation occurs. A rapid retroperitoneal air leak can spread to multiple body compartments, including the mediastinum, pleura, neck, subcutaneous tissues, scrotum, and musculature by tracking through various fascial planes. Rarely, rapid ingress of air in these areas can lead to compartment syndrome.⁴

Small perforations tend to close spontaneously and may remain clinically silent, but large or persistent perforations are known to cause subcutaneous emphysema, sepsis, and respiratory failure.⁵

Our patient’s type 2 perforation

We presumed that our patient had a type 2 perforation, given the finding of retroperitoneal air. Difficulty cannulating the biliary tree via the friable malignant tissue at the site of the major papilla likely caused punctate perforations, resulting in air leakage into the retroperitoneum. Punctate perforations typically do not allow contrast extravasation, explaining the absence of oral contrast leakage on CT.

TREATMENT OF ENDOSCOPY-RELATED PERFORATION

Conventional supine and upright abdominal radiography is an appropriate initial imaging modality to confirm the diagnosis. However, CT is more sensitive and accurate, especially when air leakage is confined to the retroperitoneum. Intravenous or oral contrast is not necessary but may help localize the perforation and better delineate fluid collections and abscesses.²

Once perforation is suspected, treatment with a broad-spectrum antibiotic, bowel rest, and stomach decompression is imperative.⁶ Further management depends on the type of perforation and the overall clinical picture. Type 1 perforations usually require immediate surgical intervention. Type 2 perforations often seal spontaneously within 2 to 3 days and thus are managed conservatively (ie, a broad-spectrum antibiotic, gastric decompression, and bowel rest), unless there is a persistent leak or a large fluid collection. Type 3 perforations rarely require surgery since most are very small and close spontaneously, and so they are managed conservatively. Type 4 perforations are the least serious. They result in retroperitoneal free air that is thought be related to the use of compressed air for lumen patency. They require only conservative measures.¹ 

A 68-year-old man with metastatic periampullary adenocarcinoma presented to his usual clinic for a scheduled biliary stent exchange by endoscopic retrograde cholangiopancreatography (ERCP). The stent had been placed 5 months before, and no complications had been reported during that procedure.

During the stent exchange procedure, the endoscopist advanced the scope to the second part of the duodenum, where a large, ulcerated, friable mass was visualized surrounding the ampulla, consistent with patient’s known periampullary cancer. The biliary stent was removed without much difficulty. However, several attempts to cannulate the common bile duct with a preloaded guidewire failed because of extensive edema and tissue friability, and to avoid further discomfort to the patient, the procedure was aborted. No perforation was visualized during or at the end of the procedure.

During the first hour after the procedure was stopped, the patient suddenly developed abdominal pain and distention and crepitus of the right chest wall. Supine abdominal radiography showed extensive pneumoperitoneum and subcutaneous emphysema in the chest. A nasogastric tube was placed for decompression, and the patient was transferred to the surgical intensive care unit at our hospital.

EVIDENCE OF PERFORATION NOTED

On arrival, the patient’s oxygen saturation was 99% while receiving oxygen at 2 L/minute by nasal cannula. The physical examination revealed neck swelling, abdominal distention, and crepitus in the neck, abdomen, scrotum, and right lower extremity.

Computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast revealed widespread pneumoretroperitoneum, pneumoperitoneum, and air along the intermuscular planes in the right lower extremity, with no evidence of extravasation of oral contrast (Figure 1). Also noted were bilateral pneumothorax, pneumomediastinum, pneumopericardium, and extensive subcutaneous emphysema (Figure 2).

Despite these impressive findings, the patient remained hemodynamically stable and was managed conservatively with broad-spectrum antibiotics, gastric decompression, and bowel rest. But repeat chest radiography 5 hours after admission to the hospital revealed an enlarging right pneumothorax, which was treated with placement of a pigtail catheter. The patient continued to improve with conservative management and was discharged on the 6th day of hospitalization.

PERFORATION DURING ERCP: INCIDENCE AND COMPLICATIONS

Although perforation is an uncommon complication of ERCP, with an incidence of 1%, mortality rates as high as 18% have been reported.¹ Older age, longer procedural time, anatomic variations, and diseases of the duodenum and common bile duct can increase the risk of perforation.²

Types of perforation

Stapfer et al¹ classified perforation during ERCP into four types, based on etiology and  site of perforation. Type 1 is perforation of the lateral or medial duodenal wall caused by excessive pressure from the endoscope or its acute angulation. Type 2 is periampullary injury, often associated with sphincterotomy or difficulty accessing the biliary tree. Type 3 is injury to the common bile duct or pancreatic duct caused by instrumentation. Type 4 is the presence of retroperitoneal free air with no evidence of actual perforation; this is usually an incidental finding and is of little or no clinical consequence.¹

In 2015, a review of 18 studies described the distribution of ERCP perforation according to the Stapfer classification: 25% were type 1, 46% were type 2, and 22% were type 3.³

Effects of air insufflation

ERCP requires air insufflation for optimal visualization. During difficult or prolonged procedures, a larger amount of air may be insufflated to maintain bowel lumen visibility. Depending on the site and size of the defect, a variable amount of air can leak under pressure once the perforation occurs. A rapid retroperitoneal air leak can spread to multiple body compartments, including the mediastinum, pleura, neck, subcutaneous tissues, scrotum, and musculature by tracking through various fascial planes. Rarely, rapid ingress of air in these areas can lead to compartment syndrome.⁴

Small perforations tend to close spontaneously and may remain clinically silent, but large or persistent perforations are known to cause subcutaneous emphysema, sepsis, and respiratory failure.⁵

Our patient’s type 2 perforation

We presumed that our patient had a type 2 perforation, given the finding of retroperitoneal air. Difficulty cannulating the biliary tree via the friable malignant tissue at the site of the major papilla likely caused punctate perforations, resulting in air leakage into the retroperitoneum. Punctate perforations typically do not allow contrast extravasation, explaining the absence of oral contrast leakage on CT.

TREATMENT OF ENDOSCOPY-RELATED PERFORATION

Conventional supine and upright abdominal radiography is an appropriate initial imaging modality to confirm the diagnosis. However, CT is more sensitive and accurate, especially when air leakage is confined to the retroperitoneum. Intravenous or oral contrast is not necessary but may help localize the perforation and better delineate fluid collections and abscesses.²

Once perforation is suspected, treatment with a broad-spectrum antibiotic, bowel rest, and stomach decompression is imperative.⁶ Further management depends on the type of perforation and the overall clinical picture. Type 1 perforations usually require immediate surgical intervention. Type 2 perforations often seal spontaneously within 2 to 3 days and thus are managed conservatively (ie, a broad-spectrum antibiotic, gastric decompression, and bowel rest), unless there is a persistent leak or a large fluid collection. Type 3 perforations rarely require surgery since most are very small and close spontaneously, and so they are managed conservatively. Type 4 perforations are the least serious. They result in retroperitoneal free air that is thought be related to the use of compressed air for lumen patency. They require only conservative measures.¹ 

A 68-year-old man with metastatic periampullary adenocarcinoma presented to his usual clinic for a scheduled biliary stent exchange by endoscopic retrograde cholangiopancreatography (ERCP). The stent had been placed 5 months before, and no complications had been reported during that procedure.

During the stent exchange procedure, the endoscopist advanced the scope to the second part of the duodenum, where a large, ulcerated, friable mass was visualized surrounding the ampulla, consistent with patient’s known periampullary cancer. The biliary stent was removed without much difficulty. However, several attempts to cannulate the common bile duct with a preloaded guidewire failed because of extensive edema and tissue friability, and to avoid further discomfort to the patient, the procedure was aborted. No perforation was visualized during or at the end of the procedure.

During the first hour after the procedure was stopped, the patient suddenly developed abdominal pain and distention and crepitus of the right chest wall. Supine abdominal radiography showed extensive pneumoperitoneum and subcutaneous emphysema in the chest. A nasogastric tube was placed for decompression, and the patient was transferred to the surgical intensive care unit at our hospital.

EVIDENCE OF PERFORATION NOTED

On arrival, the patient’s oxygen saturation was 99% while receiving oxygen at 2 L/minute by nasal cannula. The physical examination revealed neck swelling, abdominal distention, and crepitus in the neck, abdomen, scrotum, and right lower extremity.

Computed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast revealed widespread pneumoretroperitoneum, pneumoperitoneum, and air along the intermuscular planes in the right lower extremity, with no evidence of extravasation of oral contrast (Figure 1). Also noted were bilateral pneumothorax, pneumomediastinum, pneumopericardium, and extensive subcutaneous emphysema (Figure 2).

Despite these impressive findings, the patient remained hemodynamically stable and was managed conservatively with broad-spectrum antibiotics, gastric decompression, and bowel rest. But repeat chest radiography 5 hours after admission to the hospital revealed an enlarging right pneumothorax, which was treated with placement of a pigtail catheter. The patient continued to improve with conservative management and was discharged on the 6th day of hospitalization.

PERFORATION DURING ERCP: INCIDENCE AND COMPLICATIONS

Although perforation is an uncommon complication of ERCP, with an incidence of 1%, mortality rates as high as 18% have been reported.¹ Older age, longer procedural time, anatomic variations, and diseases of the duodenum and common bile duct can increase the risk of perforation.²

Types of perforation

Stapfer et al¹ classified perforation during ERCP into four types, based on etiology and  site of perforation. Type 1 is perforation of the lateral or medial duodenal wall caused by excessive pressure from the endoscope or its acute angulation. Type 2 is periampullary injury, often associated with sphincterotomy or difficulty accessing the biliary tree. Type 3 is injury to the common bile duct or pancreatic duct caused by instrumentation. Type 4 is the presence of retroperitoneal free air with no evidence of actual perforation; this is usually an incidental finding and is of little or no clinical consequence.¹

In 2015, a review of 18 studies described the distribution of ERCP perforation according to the Stapfer classification: 25% were type 1, 46% were type 2, and 22% were type 3.³

Effects of air insufflation

ERCP requires air insufflation for optimal visualization. During difficult or prolonged procedures, a larger amount of air may be insufflated to maintain bowel lumen visibility. Depending on the site and size of the defect, a variable amount of air can leak under pressure once the perforation occurs. A rapid retroperitoneal air leak can spread to multiple body compartments, including the mediastinum, pleura, neck, subcutaneous tissues, scrotum, and musculature by tracking through various fascial planes. Rarely, rapid ingress of air in these areas can lead to compartment syndrome.⁴

Small perforations tend to close spontaneously and may remain clinically silent, but large or persistent perforations are known to cause subcutaneous emphysema, sepsis, and respiratory failure.⁵

Our patient’s type 2 perforation

We presumed that our patient had a type 2 perforation, given the finding of retroperitoneal air. Difficulty cannulating the biliary tree via the friable malignant tissue at the site of the major papilla likely caused punctate perforations, resulting in air leakage into the retroperitoneum. Punctate perforations typically do not allow contrast extravasation, explaining the absence of oral contrast leakage on CT.

TREATMENT OF ENDOSCOPY-RELATED PERFORATION

Conventional supine and upright abdominal radiography is an appropriate initial imaging modality to confirm the diagnosis. However, CT is more sensitive and accurate, especially when air leakage is confined to the retroperitoneum. Intravenous or oral contrast is not necessary but may help localize the perforation and better delineate fluid collections and abscesses.²

Once perforation is suspected, treatment with a broad-spectrum antibiotic, bowel rest, and stomach decompression is imperative.⁶ Further management depends on the type of perforation and the overall clinical picture. Type 1 perforations usually require immediate surgical intervention. Type 2 perforations often seal spontaneously within 2 to 3 days and thus are managed conservatively (ie, a broad-spectrum antibiotic, gastric decompression, and bowel rest), unless there is a persistent leak or a large fluid collection. Type 3 perforations rarely require surgery since most are very small and close spontaneously, and so they are managed conservatively. Type 4 perforations are the least serious. They result in retroperitoneal free air that is thought be related to the use of compressed air for lumen patency. They require only conservative measures.¹ 

Over the summer, I lectured in an internal medicine maintenance-of-certification course. Using a case-based discussion format, I presented real patients to review the breadth of rheumatic diseases that the course participants might encounter in their practices and on board examinations. At a break, one of the participants, obviously frustrated, approached me to say that he thought that I had mentioned too many uncommon situations, that he didn’t need to know secondary treatment options and disease mimics—I should just have reviewed the common aspects of the common diseases he would encounter in practice (and on the exam).

I was a bit taken aback. I try hard to avoid emphasizing the arcane details that only a subspecialist needs to know. I reconsidered my approach: should I only review the common and the expected? But without knowing something about the clinical permutations and the mimics of the common diseases we encounter, I don’t know how we can avoid making incorrect diagnoses due to the cognitive bias of familiarity. Common diseases are indeed common, but that doesn’t mean patients with less-common ones don’t find their way into our practices. As internists, we need to constantly humble ourselves with the knowledge that there is much in medicine, in every specialty, that we can’t remember, do not recognize, or just don’t know enough about. It is not just about “what will be on the test.” When a patient exhibits atypical features of the common disease that they have been diagnosed with, or doesn’t respond as expected to therapy, we need to remember that less-common mimics of that disease exist, even if we can’t remember the specific names or the pathophysiology.

Peripheral vascular disease is common, is rarely diagnosed early, and is usually related to atherosclerosis. Significant disease is generally manifested by symptoms of exertional limb ischemia or even pain at rest and stigmata of distal tissue hypoxia and necrosis from the decreased supply of oxygen and nutrients. But there are nonatherosclerotic causes of limb claudication, distal ischemia, and livedo reticularis. In my clinic, patients with those symptoms and findings are quite likely to have a form of large vessel arteritis, Raynaud phenomenon, or both. In a patient with Takayasu or giant cell arteritis, pain with repetitive arm use is more likely from subclavian occlusion than rotator cuff disease, whereas the latter is the far more common cause of arm pain with repetitive motion when patients see a physician for “arm pain” before the diagnosis of arteritis is considered. We need to entertain alternative diagnoses in order to perform the appropriate physical examination, obtain appropriate diagnostic studies, and initiate the correct therapy.

Eun et al, in this issue of the Journal, highlight and briefly discuss nine nonatherosclerotic entities that cause limb ischemia. The diagnostic assessment of these can be invasive and expensive, yet may be warranted to provide the appropriate therapy. The diagnosis not pursued will rarely be made in a timely manner, and the diagnosis not considered will not be pursued at all. And with these diseases, the patient’s history and examination usually provide the clues that the horse has stripes.

Eun et al highlight clinical features that may distinguish these entities from typical atherosclerotic peripheral vascular disease. Recognizing the features that are atypical for atherosclerosis implies that we know the more typical features of the disease. Knowing something about the uncommon as well as the common helps prepare us for the real test—the patient in front of us. And that patient doesn’t usually know that he or she is a zebra and not a horse.

Last month, I highlighted some staff changes in our editorial staff. I want to take this opportunity to introduce Craig Nielsen, MD, as our new deputy editor. Craig is a superb internist in both inpatient and outpatient arenas, a medical educator, and former director of our internal medicine residency program.

Over the summer, I lectured in an internal medicine maintenance-of-certification course. Using a case-based discussion format, I presented real patients to review the breadth of rheumatic diseases that the course participants might encounter in their practices and on board examinations. At a break, one of the participants, obviously frustrated, approached me to say that he thought that I had mentioned too many uncommon situations, that he didn’t need to know secondary treatment options and disease mimics—I should just have reviewed the common aspects of the common diseases he would encounter in practice (and on the exam).

I was a bit taken aback. I try hard to avoid emphasizing the arcane details that only a subspecialist needs to know. I reconsidered my approach: should I only review the common and the expected? But without knowing something about the clinical permutations and the mimics of the common diseases we encounter, I don’t know how we can avoid making incorrect diagnoses due to the cognitive bias of familiarity. Common diseases are indeed common, but that doesn’t mean patients with less-common ones don’t find their way into our practices. As internists, we need to constantly humble ourselves with the knowledge that there is much in medicine, in every specialty, that we can’t remember, do not recognize, or just don’t know enough about. It is not just about “what will be on the test.” When a patient exhibits atypical features of the common disease that they have been diagnosed with, or doesn’t respond as expected to therapy, we need to remember that less-common mimics of that disease exist, even if we can’t remember the specific names or the pathophysiology.

Peripheral vascular disease is common, is rarely diagnosed early, and is usually related to atherosclerosis. Significant disease is generally manifested by symptoms of exertional limb ischemia or even pain at rest and stigmata of distal tissue hypoxia and necrosis from the decreased supply of oxygen and nutrients. But there are nonatherosclerotic causes of limb claudication, distal ischemia, and livedo reticularis. In my clinic, patients with those symptoms and findings are quite likely to have a form of large vessel arteritis, Raynaud phenomenon, or both. In a patient with Takayasu or giant cell arteritis, pain with repetitive arm use is more likely from subclavian occlusion than rotator cuff disease, whereas the latter is the far more common cause of arm pain with repetitive motion when patients see a physician for “arm pain” before the diagnosis of arteritis is considered. We need to entertain alternative diagnoses in order to perform the appropriate physical examination, obtain appropriate diagnostic studies, and initiate the correct therapy.

Eun et al, in this issue of the Journal, highlight and briefly discuss nine nonatherosclerotic entities that cause limb ischemia. The diagnostic assessment of these can be invasive and expensive, yet may be warranted to provide the appropriate therapy. The diagnosis not pursued will rarely be made in a timely manner, and the diagnosis not considered will not be pursued at all. And with these diseases, the patient’s history and examination usually provide the clues that the horse has stripes.

Eun et al highlight clinical features that may distinguish these entities from typical atherosclerotic peripheral vascular disease. Recognizing the features that are atypical for atherosclerosis implies that we know the more typical features of the disease. Knowing something about the uncommon as well as the common helps prepare us for the real test—the patient in front of us. And that patient doesn’t usually know that he or she is a zebra and not a horse.

Last month, I highlighted some staff changes in our editorial staff. I want to take this opportunity to introduce Craig Nielsen, MD, as our new deputy editor. Craig is a superb internist in both inpatient and outpatient arenas, a medical educator, and former director of our internal medicine residency program.

Over the summer, I lectured in an internal medicine maintenance-of-certification course. Using a case-based discussion format, I presented real patients to review the breadth of rheumatic diseases that the course participants might encounter in their practices and on board examinations. At a break, one of the participants, obviously frustrated, approached me to say that he thought that I had mentioned too many uncommon situations, that he didn’t need to know secondary treatment options and disease mimics—I should just have reviewed the common aspects of the common diseases he would encounter in practice (and on the exam).

I was a bit taken aback. I try hard to avoid emphasizing the arcane details that only a subspecialist needs to know. I reconsidered my approach: should I only review the common and the expected? But without knowing something about the clinical permutations and the mimics of the common diseases we encounter, I don’t know how we can avoid making incorrect diagnoses due to the cognitive bias of familiarity. Common diseases are indeed common, but that doesn’t mean patients with less-common ones don’t find their way into our practices. As internists, we need to constantly humble ourselves with the knowledge that there is much in medicine, in every specialty, that we can’t remember, do not recognize, or just don’t know enough about. It is not just about “what will be on the test.” When a patient exhibits atypical features of the common disease that they have been diagnosed with, or doesn’t respond as expected to therapy, we need to remember that less-common mimics of that disease exist, even if we can’t remember the specific names or the pathophysiology.

Peripheral vascular disease is common, is rarely diagnosed early, and is usually related to atherosclerosis. Significant disease is generally manifested by symptoms of exertional limb ischemia or even pain at rest and stigmata of distal tissue hypoxia and necrosis from the decreased supply of oxygen and nutrients. But there are nonatherosclerotic causes of limb claudication, distal ischemia, and livedo reticularis. In my clinic, patients with those symptoms and findings are quite likely to have a form of large vessel arteritis, Raynaud phenomenon, or both. In a patient with Takayasu or giant cell arteritis, pain with repetitive arm use is more likely from subclavian occlusion than rotator cuff disease, whereas the latter is the far more common cause of arm pain with repetitive motion when patients see a physician for “arm pain” before the diagnosis of arteritis is considered. We need to entertain alternative diagnoses in order to perform the appropriate physical examination, obtain appropriate diagnostic studies, and initiate the correct therapy.

Eun et al, in this issue of the Journal, highlight and briefly discuss nine nonatherosclerotic entities that cause limb ischemia. The diagnostic assessment of these can be invasive and expensive, yet may be warranted to provide the appropriate therapy. The diagnosis not pursued will rarely be made in a timely manner, and the diagnosis not considered will not be pursued at all. And with these diseases, the patient’s history and examination usually provide the clues that the horse has stripes.

Eun et al highlight clinical features that may distinguish these entities from typical atherosclerotic peripheral vascular disease. Recognizing the features that are atypical for atherosclerosis implies that we know the more typical features of the disease. Knowing something about the uncommon as well as the common helps prepare us for the real test—the patient in front of us. And that patient doesn’t usually know that he or she is a zebra and not a horse.

Last month, I highlighted some staff changes in our editorial staff. I want to take this opportunity to introduce Craig Nielsen, MD, as our new deputy editor. Craig is a superb internist in both inpatient and outpatient arenas, a medical educator, and former director of our internal medicine residency program.

LONDON – Higher levels of a neuronal protein were found in the blood of patients with relapsing-remitting multiple sclerosis than in healthy subjects in a proof-of-concept study reported at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS).

Blood levels of neurofilament light chain (NfL) were 28.1 vs. 12.5 pg/mL, respectively (P less than .0001), and were also found to be higher in relapsing-remitting multiple sclerosis (RRMS) patients with greater disease activity seen on MRI.

LONDON – Higher levels of a neuronal protein were found in the blood of patients with relapsing-remitting multiple sclerosis than in healthy subjects in a proof-of-concept study reported at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS).

Blood levels of neurofilament light chain (NfL) were 28.1 vs. 12.5 pg/mL, respectively (P less than .0001), and were also found to be higher in relapsing-remitting multiple sclerosis (RRMS) patients with greater disease activity seen on MRI.

LONDON – Higher levels of a neuronal protein were found in the blood of patients with relapsing-remitting multiple sclerosis than in healthy subjects in a proof-of-concept study reported at the annual congress of the European Committee for Treatment and Research in Multiple Sclerosis (ECTRIMS).

Blood levels of neurofilament light chain (NfL) were 28.1 vs. 12.5 pg/mL, respectively (P less than .0001), and were also found to be higher in relapsing-remitting multiple sclerosis (RRMS) patients with greater disease activity seen on MRI.

BY CATALINA MATIZ, MD, AND ANDREA WALDMAN, MD

At the time of initial evaluation, the patient was prescribed a 2-week course of topical triamcinolone 0.1% ointment. Subsequently, the cutaneous lesions clinically improved, but did not completely resolve. Mother had no recollection of infant contact with metal, plastic, or additional topical emollients or medications. On further investigation, the patient’s mother revealed the recent conversion to a different brand of diaper. The diapers had a blue and green polka dot design corresponding with the distribution of our patient’s cutaneous lesions, highly suggestive of allergic contact dermatitis (ACD).

Diaper dermatitis represents a common complaint peaking at 9-12 months of age, estimated to occur in 25% of infants in the primary care office setting.1 Irritant contact dermatitis (ICD) predominates as the most common etiology of diaper dermatitis. ICD represents a nonimmunologic response, triggered by maceration and breakdown of the cutaneous barrier, directly resulting from chronic exposure to urine and feces.1 In contrast, ACD characterizes an immunologic reaction to specific offending allergen(s).

ACD can arise in the diaper area following contact with dyes, fragrances, or other constituents of disposable diapers, wet wipes, diaper creams, or medications. Alberta et al. initially described ACD in several infants, resulting from blue, green, and pink dyes present in various disposable diapers. The dermatitis promptly resolved upon switching to dye-free diapers.2 Furthermore, topical products applied to the diaper region such as barrier creams and wet wipes may trigger allergic sensitization. Sensitization to an allergen can occur after few exposures to years after introduction. Following the initial contact reaction, re-exposure to the offending allergen can elicit a quicker cutaneous response. Case reports have detailed cases of ACD presenting in neonates as early as 1 week of age.3

If ACD is suspected as an etiologic factor in diaper dermatitis, providers should consider constituents present in topical formulations, wet wipes, and components of the diaper itself. A specific example includes mercaptobenzothiazole, a rubber additive commonly found in elastic bands of disposable diapers.2 The most common potential allergens discovered in a recent review of 63 diaper wipes, 41 topical diaper preparations, and 3 top-selling diaper brands included botanical extracts (i.e. aloe vera), alpha-tocopherol, fragrances, propylene glycol, parabens, iodopropynyl butylcarbamate, and lanolin.4

ACD may be difficult to diagnose. Obtaining a thorough clinical history is essential to diagnosis. ACD is often distinguished by its distinct cutaneous morphology, with initial lesions commonly characterized by sharply demarcated erythema associated with vesiculation and edema. The dermatitis progressively evolves to a mixed presentation of predominantly eczematous papules and plaques with crusting, scaling, and lichenification. Another clue suggestive of ACD is cutaneous involvement limited to areas of allergen contact, such as the geometric plaques corresponding to areas of skin exposure to blue and green dye in our patient.1

Differential diagnosis

In contrast to ACD, ICD commonly erupts in the perianal region in the presence of feces, especially during diarrheal episodes. Common clinical manifestations include skin maceration, erosions, erythema, papulation, and scaling. More severe presentations of irritant dermatitis include Jacquet’s diaper dermatitis, perianal pseudoverrucous papules and nodules and granuloma gluteale infantum.1 These cutaneous findings also may develop in older patients with irritated peristomal skin.5

Inflammatory dermatoses such as psoriasis and seborrheic dermatitis also may erupt in the diaper region. Psoriasis commonly involves the gluteal cleft and inguinal folds, appearing as well-demarcated, pink plaques with thin white scale.5 Persistence of plaques despite topical corticosteroid application and lack of pruritus further delineates this disorder. Family history of psoriasis may provide an additional clue to the diagnosis of psoriasis. Similarly, seborrheic dermatitis may present with diaper involvement characterized by well-demarcated erythematous plaques with minimal or absent greasy yellow scale. This dermatitis commonly erupts in infants and neonates less than 6 weeks old and resolves by 6-9 months of age.1

Langerhans cell histiocytosis (LCH) represents a serious condition important to consider in the differential diagnosis of persistent recalcitrant diaper dermatitis. Often confused with seborrheic dermatitis, the diaper dermatitis of LCH may be differentiated by petechiae, purpura, and/or ulceration and atrophy of the inguinal folds.5

Management

Avoidance of the allergen constitutes the gold standard of treatment for ACD. Initial treatment involves adequate-potency topical corticosteroids; for the diaper area, low- to mid-potency corticosteroids are recommended. Changing to dye-free diapers and/or avoiding contact with the suspected allergen also is advised. Epicutaneous patch testing is recommended if the dermatitis fails to improve despite removal of the suspected offending agent.5

References

1. Pediatr Dermatol. 2014 Nov;31 Suppl 1:19-24.

2. Pediatrics. 2005 Sep;116(3):e450-2.

3. Cutis. 1994 Nov;54(5):300-2.

4. Dermatitis. 2016 May-Jun;27(3):110-8.

5. “Neonatal and Infant Dermatology,” 3rd edition (Philadelphia: Elsevier, 2015, p. 251)

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Dr. Waldman is a clinical research fellow at the hospital. Email them at [email protected].

BY CATALINA MATIZ, MD, AND ANDREA WALDMAN, MD

At the time of initial evaluation, the patient was prescribed a 2-week course of topical triamcinolone 0.1% ointment. Subsequently, the cutaneous lesions clinically improved, but did not completely resolve. Mother had no recollection of infant contact with metal, plastic, or additional topical emollients or medications. On further investigation, the patient’s mother revealed the recent conversion to a different brand of diaper. The diapers had a blue and green polka dot design corresponding with the distribution of our patient’s cutaneous lesions, highly suggestive of allergic contact dermatitis (ACD).

Diaper dermatitis represents a common complaint peaking at 9-12 months of age, estimated to occur in 25% of infants in the primary care office setting.1 Irritant contact dermatitis (ICD) predominates as the most common etiology of diaper dermatitis. ICD represents a nonimmunologic response, triggered by maceration and breakdown of the cutaneous barrier, directly resulting from chronic exposure to urine and feces.1 In contrast, ACD characterizes an immunologic reaction to specific offending allergen(s).

ACD can arise in the diaper area following contact with dyes, fragrances, or other constituents of disposable diapers, wet wipes, diaper creams, or medications. Alberta et al. initially described ACD in several infants, resulting from blue, green, and pink dyes present in various disposable diapers. The dermatitis promptly resolved upon switching to dye-free diapers.2 Furthermore, topical products applied to the diaper region such as barrier creams and wet wipes may trigger allergic sensitization. Sensitization to an allergen can occur after few exposures to years after introduction. Following the initial contact reaction, re-exposure to the offending allergen can elicit a quicker cutaneous response. Case reports have detailed cases of ACD presenting in neonates as early as 1 week of age.3

If ACD is suspected as an etiologic factor in diaper dermatitis, providers should consider constituents present in topical formulations, wet wipes, and components of the diaper itself. A specific example includes mercaptobenzothiazole, a rubber additive commonly found in elastic bands of disposable diapers.2 The most common potential allergens discovered in a recent review of 63 diaper wipes, 41 topical diaper preparations, and 3 top-selling diaper brands included botanical extracts (i.e. aloe vera), alpha-tocopherol, fragrances, propylene glycol, parabens, iodopropynyl butylcarbamate, and lanolin.4

ACD may be difficult to diagnose. Obtaining a thorough clinical history is essential to diagnosis. ACD is often distinguished by its distinct cutaneous morphology, with initial lesions commonly characterized by sharply demarcated erythema associated with vesiculation and edema. The dermatitis progressively evolves to a mixed presentation of predominantly eczematous papules and plaques with crusting, scaling, and lichenification. Another clue suggestive of ACD is cutaneous involvement limited to areas of allergen contact, such as the geometric plaques corresponding to areas of skin exposure to blue and green dye in our patient.1

Differential diagnosis

In contrast to ACD, ICD commonly erupts in the perianal region in the presence of feces, especially during diarrheal episodes. Common clinical manifestations include skin maceration, erosions, erythema, papulation, and scaling. More severe presentations of irritant dermatitis include Jacquet’s diaper dermatitis, perianal pseudoverrucous papules and nodules and granuloma gluteale infantum.1 These cutaneous findings also may develop in older patients with irritated peristomal skin.5

Inflammatory dermatoses such as psoriasis and seborrheic dermatitis also may erupt in the diaper region. Psoriasis commonly involves the gluteal cleft and inguinal folds, appearing as well-demarcated, pink plaques with thin white scale.5 Persistence of plaques despite topical corticosteroid application and lack of pruritus further delineates this disorder. Family history of psoriasis may provide an additional clue to the diagnosis of psoriasis. Similarly, seborrheic dermatitis may present with diaper involvement characterized by well-demarcated erythematous plaques with minimal or absent greasy yellow scale. This dermatitis commonly erupts in infants and neonates less than 6 weeks old and resolves by 6-9 months of age.1

Langerhans cell histiocytosis (LCH) represents a serious condition important to consider in the differential diagnosis of persistent recalcitrant diaper dermatitis. Often confused with seborrheic dermatitis, the diaper dermatitis of LCH may be differentiated by petechiae, purpura, and/or ulceration and atrophy of the inguinal folds.5

Management

Avoidance of the allergen constitutes the gold standard of treatment for ACD. Initial treatment involves adequate-potency topical corticosteroids; for the diaper area, low- to mid-potency corticosteroids are recommended. Changing to dye-free diapers and/or avoiding contact with the suspected allergen also is advised. Epicutaneous patch testing is recommended if the dermatitis fails to improve despite removal of the suspected offending agent.5

References

1. Pediatr Dermatol. 2014 Nov;31 Suppl 1:19-24.

2. Pediatrics. 2005 Sep;116(3):e450-2.

3. Cutis. 1994 Nov;54(5):300-2.

4. Dermatitis. 2016 May-Jun;27(3):110-8.

5. “Neonatal and Infant Dermatology,” 3rd edition (Philadelphia: Elsevier, 2015, p. 251)

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Dr. Waldman is a clinical research fellow at the hospital. Email them at [email protected].

BY CATALINA MATIZ, MD, AND ANDREA WALDMAN, MD

At the time of initial evaluation, the patient was prescribed a 2-week course of topical triamcinolone 0.1% ointment. Subsequently, the cutaneous lesions clinically improved, but did not completely resolve. Mother had no recollection of infant contact with metal, plastic, or additional topical emollients or medications. On further investigation, the patient’s mother revealed the recent conversion to a different brand of diaper. The diapers had a blue and green polka dot design corresponding with the distribution of our patient’s cutaneous lesions, highly suggestive of allergic contact dermatitis (ACD).

Diaper dermatitis represents a common complaint peaking at 9-12 months of age, estimated to occur in 25% of infants in the primary care office setting.1 Irritant contact dermatitis (ICD) predominates as the most common etiology of diaper dermatitis. ICD represents a nonimmunologic response, triggered by maceration and breakdown of the cutaneous barrier, directly resulting from chronic exposure to urine and feces.1 In contrast, ACD characterizes an immunologic reaction to specific offending allergen(s).

ACD can arise in the diaper area following contact with dyes, fragrances, or other constituents of disposable diapers, wet wipes, diaper creams, or medications. Alberta et al. initially described ACD in several infants, resulting from blue, green, and pink dyes present in various disposable diapers. The dermatitis promptly resolved upon switching to dye-free diapers.2 Furthermore, topical products applied to the diaper region such as barrier creams and wet wipes may trigger allergic sensitization. Sensitization to an allergen can occur after few exposures to years after introduction. Following the initial contact reaction, re-exposure to the offending allergen can elicit a quicker cutaneous response. Case reports have detailed cases of ACD presenting in neonates as early as 1 week of age.3

If ACD is suspected as an etiologic factor in diaper dermatitis, providers should consider constituents present in topical formulations, wet wipes, and components of the diaper itself. A specific example includes mercaptobenzothiazole, a rubber additive commonly found in elastic bands of disposable diapers.2 The most common potential allergens discovered in a recent review of 63 diaper wipes, 41 topical diaper preparations, and 3 top-selling diaper brands included botanical extracts (i.e. aloe vera), alpha-tocopherol, fragrances, propylene glycol, parabens, iodopropynyl butylcarbamate, and lanolin.4

ACD may be difficult to diagnose. Obtaining a thorough clinical history is essential to diagnosis. ACD is often distinguished by its distinct cutaneous morphology, with initial lesions commonly characterized by sharply demarcated erythema associated with vesiculation and edema. The dermatitis progressively evolves to a mixed presentation of predominantly eczematous papules and plaques with crusting, scaling, and lichenification. Another clue suggestive of ACD is cutaneous involvement limited to areas of allergen contact, such as the geometric plaques corresponding to areas of skin exposure to blue and green dye in our patient.1

Differential diagnosis

In contrast to ACD, ICD commonly erupts in the perianal region in the presence of feces, especially during diarrheal episodes. Common clinical manifestations include skin maceration, erosions, erythema, papulation, and scaling. More severe presentations of irritant dermatitis include Jacquet’s diaper dermatitis, perianal pseudoverrucous papules and nodules and granuloma gluteale infantum.1 These cutaneous findings also may develop in older patients with irritated peristomal skin.5

Inflammatory dermatoses such as psoriasis and seborrheic dermatitis also may erupt in the diaper region. Psoriasis commonly involves the gluteal cleft and inguinal folds, appearing as well-demarcated, pink plaques with thin white scale.5 Persistence of plaques despite topical corticosteroid application and lack of pruritus further delineates this disorder. Family history of psoriasis may provide an additional clue to the diagnosis of psoriasis. Similarly, seborrheic dermatitis may present with diaper involvement characterized by well-demarcated erythematous plaques with minimal or absent greasy yellow scale. This dermatitis commonly erupts in infants and neonates less than 6 weeks old and resolves by 6-9 months of age.1

Langerhans cell histiocytosis (LCH) represents a serious condition important to consider in the differential diagnosis of persistent recalcitrant diaper dermatitis. Often confused with seborrheic dermatitis, the diaper dermatitis of LCH may be differentiated by petechiae, purpura, and/or ulceration and atrophy of the inguinal folds.5

Management

Avoidance of the allergen constitutes the gold standard of treatment for ACD. Initial treatment involves adequate-potency topical corticosteroids; for the diaper area, low- to mid-potency corticosteroids are recommended. Changing to dye-free diapers and/or avoiding contact with the suspected allergen also is advised. Epicutaneous patch testing is recommended if the dermatitis fails to improve despite removal of the suspected offending agent.5

References

1. Pediatr Dermatol. 2014 Nov;31 Suppl 1:19-24.

2. Pediatrics. 2005 Sep;116(3):e450-2.

3. Cutis. 1994 Nov;54(5):300-2.

4. Dermatitis. 2016 May-Jun;27(3):110-8.

5. “Neonatal and Infant Dermatology,” 3rd edition (Philadelphia: Elsevier, 2015, p. 251)

Dr. Matiz is assistant professor of dermatology at Rady Children’s Hospital San Diego–University of California, San Diego, and Dr. Waldman is a clinical research fellow at the hospital. Email them at [email protected].

A new practice guideline from the Endocrine Society recommends continuous glucose monitoring as the new standard for tracking blood sugar in adults with type 1 diabetes.

“Studies have found that people with type 1 diabetes who use CGMs [continuous glucose monitors] are able to maintain better control of their blood sugar without increasing episodes of hypoglycemia when blood sugar drops to dangerous levels, compared to those who self-monitor blood glucose with periodic finger sticks,” the chair of the guideline task force, Anne Peters, MD, a professor of medicine at the University of Southern California, Los Angeles, said in a Sept. 26 statement.

The group recommended CGMs for well-controlled type 1 patients as well as those above hemoglobin A_1c (HbA_1c) targets, so long as they want and understand how to use the devices. It also suggested short-term, intermittent CGM use to help type 2 patients meet HbA_1cgoals. Insulin pumps were recommended over multiple daily injections for type 1 patients above target HbA_1clevels, as well as those who meet their target but continue to have severe hypoglycemia or high glucose variability. For patients with type 2 disease, pumps were suggested for cases of poor glycemic control despite intensive insulin therapy, oral agents, and other measures.

The Endocrine Society and others have been pushing Medicare to cover CGMs for a while; the new guideline seems to support the effort. Although the devices are used by type 1 patients and covered by some insurance plans, they are only indicated as finger-stick adjuncts, not replacements. For Medicare coverage, they would “need to serve a primary medical purpose and not be used adjunctively,” according to a recent review by Dexcom, a company seeking a primary monitoring indication for its CGM.

The Endocrine Society noted in its evidence-based guideline that standard capillary blood glucose measurements “offer only a limited perspective on the constant daily changes in blood glucose levels,” and, unlike continuous monitoring, “do not provide alarms that indicate when blood glucose levels are above or below various thresholds, and do not indicate trends in blood glucose levels.”

The society commissioned a pooled analysis of 11 randomized trials that showed a 0.3% reduction in HbA_1c with real-time glucose monitoring, mostly in patients 15 years or older. Other studies cited by the group also showed better HbA_1c control than with finger sticks, without an increased risk of hypoglycemia.

The guideline also suggested insulin pumps for type 1 patients who want greater flexibility and convenience and that insulin pump therapy should continue during hospitalizations. It also suggested encouraging patients to use the embedded bolus calculators in their pumps so long as they “have appropriate education regarding their use and limitations.”

The Endocrine Society funded the work, with cosponsorship from the American Association for Clinical Chemistry, the American Association of Diabetes Educators, and the European Society of Endocrinology. Several of the authors have industry ties to companies that make CGMs or insulin pumps. Dr. Peters is an advisor for Abbott, Becton Dickinson, AstraZeneca, Biodel, Medtronic, and other companies, as well as a speaker, investigator, and advisor for Janssen.

[email protected]

A new practice guideline from the Endocrine Society recommends continuous glucose monitoring as the new standard for tracking blood sugar in adults with type 1 diabetes.

“Studies have found that people with type 1 diabetes who use CGMs [continuous glucose monitors] are able to maintain better control of their blood sugar without increasing episodes of hypoglycemia when blood sugar drops to dangerous levels, compared to those who self-monitor blood glucose with periodic finger sticks,” the chair of the guideline task force, Anne Peters, MD, a professor of medicine at the University of Southern California, Los Angeles, said in a Sept. 26 statement.

The group recommended CGMs for well-controlled type 1 patients as well as those above hemoglobin A_1c (HbA_1c) targets, so long as they want and understand how to use the devices. It also suggested short-term, intermittent CGM use to help type 2 patients meet HbA_1cgoals. Insulin pumps were recommended over multiple daily injections for type 1 patients above target HbA_1clevels, as well as those who meet their target but continue to have severe hypoglycemia or high glucose variability. For patients with type 2 disease, pumps were suggested for cases of poor glycemic control despite intensive insulin therapy, oral agents, and other measures.

The Endocrine Society and others have been pushing Medicare to cover CGMs for a while; the new guideline seems to support the effort. Although the devices are used by type 1 patients and covered by some insurance plans, they are only indicated as finger-stick adjuncts, not replacements. For Medicare coverage, they would “need to serve a primary medical purpose and not be used adjunctively,” according to a recent review by Dexcom, a company seeking a primary monitoring indication for its CGM.

The Endocrine Society noted in its evidence-based guideline that standard capillary blood glucose measurements “offer only a limited perspective on the constant daily changes in blood glucose levels,” and, unlike continuous monitoring, “do not provide alarms that indicate when blood glucose levels are above or below various thresholds, and do not indicate trends in blood glucose levels.”

The society commissioned a pooled analysis of 11 randomized trials that showed a 0.3% reduction in HbA_1c with real-time glucose monitoring, mostly in patients 15 years or older. Other studies cited by the group also showed better HbA_1c control than with finger sticks, without an increased risk of hypoglycemia.

The guideline also suggested insulin pumps for type 1 patients who want greater flexibility and convenience and that insulin pump therapy should continue during hospitalizations. It also suggested encouraging patients to use the embedded bolus calculators in their pumps so long as they “have appropriate education regarding their use and limitations.”

The Endocrine Society funded the work, with cosponsorship from the American Association for Clinical Chemistry, the American Association of Diabetes Educators, and the European Society of Endocrinology. Several of the authors have industry ties to companies that make CGMs or insulin pumps. Dr. Peters is an advisor for Abbott, Becton Dickinson, AstraZeneca, Biodel, Medtronic, and other companies, as well as a speaker, investigator, and advisor for Janssen.

[email protected]

A new practice guideline from the Endocrine Society recommends continuous glucose monitoring as the new standard for tracking blood sugar in adults with type 1 diabetes.

“Studies have found that people with type 1 diabetes who use CGMs [continuous glucose monitors] are able to maintain better control of their blood sugar without increasing episodes of hypoglycemia when blood sugar drops to dangerous levels, compared to those who self-monitor blood glucose with periodic finger sticks,” the chair of the guideline task force, Anne Peters, MD, a professor of medicine at the University of Southern California, Los Angeles, said in a Sept. 26 statement.

The group recommended CGMs for well-controlled type 1 patients as well as those above hemoglobin A_1c (HbA_1c) targets, so long as they want and understand how to use the devices. It also suggested short-term, intermittent CGM use to help type 2 patients meet HbA_1cgoals. Insulin pumps were recommended over multiple daily injections for type 1 patients above target HbA_1clevels, as well as those who meet their target but continue to have severe hypoglycemia or high glucose variability. For patients with type 2 disease, pumps were suggested for cases of poor glycemic control despite intensive insulin therapy, oral agents, and other measures.

The Endocrine Society and others have been pushing Medicare to cover CGMs for a while; the new guideline seems to support the effort. Although the devices are used by type 1 patients and covered by some insurance plans, they are only indicated as finger-stick adjuncts, not replacements. For Medicare coverage, they would “need to serve a primary medical purpose and not be used adjunctively,” according to a recent review by Dexcom, a company seeking a primary monitoring indication for its CGM.

The Endocrine Society noted in its evidence-based guideline that standard capillary blood glucose measurements “offer only a limited perspective on the constant daily changes in blood glucose levels,” and, unlike continuous monitoring, “do not provide alarms that indicate when blood glucose levels are above or below various thresholds, and do not indicate trends in blood glucose levels.”

The society commissioned a pooled analysis of 11 randomized trials that showed a 0.3% reduction in HbA_1c with real-time glucose monitoring, mostly in patients 15 years or older. Other studies cited by the group also showed better HbA_1c control than with finger sticks, without an increased risk of hypoglycemia.

The guideline also suggested insulin pumps for type 1 patients who want greater flexibility and convenience and that insulin pump therapy should continue during hospitalizations. It also suggested encouraging patients to use the embedded bolus calculators in their pumps so long as they “have appropriate education regarding their use and limitations.”

The Endocrine Society funded the work, with cosponsorship from the American Association for Clinical Chemistry, the American Association of Diabetes Educators, and the European Society of Endocrinology. Several of the authors have industry ties to companies that make CGMs or insulin pumps. Dr. Peters is an advisor for Abbott, Becton Dickinson, AstraZeneca, Biodel, Medtronic, and other companies, as well as a speaker, investigator, and advisor for Janssen.

[email protected]