Lipodystrophy in patients with juvenile dermatomyositis--evaluation of clinical and metabolic abnormalities.
OBJECTIVE
Lipodystrophy and associated metabolic abnormalities are being increasingly 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 JDM.


METHODS
Twenty patients with JDM were evaluated for evidence of lipodystrophy and associated lipoatrophic diabetes mellitus. All patients underwent clinical assessment, laboratory investigations, and metabolic studies (oral glucose tolerance test, lipid studies, insulin antibodies).


RESULTS
We found clinical evidence of lipodystrophy and lipoatrophic diabetes 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 impaired glucose tolerance (2 patients); Group 2 (Patients 5-12): no lipodystrophy but abnormal glucose and/or lipid studies; Group 3 (Patients 13-20): no lipodystrophy and no abnormalities of glucose and lipid studies.


CONCLUSIONS
We found 25% of patients with JDM have lipodystrophy, and 50% present with hypertriglyceridemia and insulin resistance. Screening for metabolic abnormalities in JDM should be included in routine followup because of the effect of lipodystrophy on longterm prognosis.
Huemer
C
C
Department of Pediatrics, University of British Columbia, Vancouver, Canada.
Kitson
H
H
Malleson
P N
PN
Sanderson
S
S
Huemer
M
M
Cabral
D A
DA
Chanoine
J P
JP
Petty
R E
RE
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D001786:Blood Glucose; D002648:Child; D002675:Child, Preschool; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D003882:Dermatomyositis / Q000453:epidemiology* / Q000378:metabolism*; D003922:Diabetes Mellitus, Type 1 / Q000175:diagnosis / Q000453:epidemiology* / Q000378:metabolism*; D005260:Female; D005951:Glucose Tolerance Test; D006801:Humans; D006946:Hyperinsulinism / Q000453:epidemiology / Q000378:metabolism; D007328:Insulin / Q000097:blood / Q000276:immunology; D007333:Insulin Resistance; D008060:Lipodystrophy / Q000453:epidemiology* / Q000378:metabolism*; D008297:Male; D015995:Prevalence; D014280:Triglycerides / Q000097:blood
D001323:Autoantibodies; D001786:Blood Glucose; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D007328:Insulin; D014280:Triglycerides