Lipodystrophy in patients with juvenile dermatomyositis - Evaluation of clinical and metabolic abnormalities

Objective. Lipodystrophy and associated metabolic abnormalities are being i ncreasingly recognized as complications of juvenile dermatomyositis (JDM). We investigated the prevalence of lipodystrophy and the extent of metabolic abnormalities related to lipoatrophic diabetes mellitus in patients with J DM. Methods. Twenty patients with JDM were evaluated for evidence of lipodystro phy and associated lipoatrophic diabetes mellitus. All patients underwent c linical assessment, laboratory investigations, and metabolic studies (oral glucose tolerance test, lipid studies, insulin antibodies). Results, We found clinical evidence of Lipodystrophy and lipoatrophic diabe tes mellitus in 4 of 20 patients with JDM and metabolic abnormalities known to be associated with lipodystrophy in another 8 patients. The 20 patients with JDM were categorized as follows: Group 1 (Patients 1-4) consisted of patients with lipodystrophy and either diabetes mellitus (2 patients) or im paired glucose tolerance (2 patients); Group 2 (Patients 5-12): no lipodyst rophy but abnormal glucose and/or lipid studies; Group 3 (Patients 13-20): no lipodystrophy and no abnormalities of glucose and lipid studies. Conclusion, We found 25% of patients with JDM have lipodystrophy, and 50% p resent with hypertriglyceridemia and insulin resistance. Screening for meta bolic abnormalities in JDM should be included in routine followup because o f the effect of lipodystrophy on longterm prognosis.