Medical College of Wisconsin
CTSICores SearchResearch InformaticsREDCap

Predictors of acquired lipodystrophy in juvenile-onset dermatomyositis and a gradient of severity. Medicine (Baltimore) 2008 Mar;87(2):70-86

Date

03/18/2008

Pubmed ID

18344805

Pubmed Central ID

PMC2674585

DOI

10.1097/MD.0b013e31816bc604

Abstract

We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. LD onset was often delayed, beginning a median of 4.6 years after diagnosis of DM. Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD. Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, a newly described myositis-associated autoantibody, was more associated with generalized LD. Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatohepatitis were frequent in patients with LD, in a gradient of frequency and severity among the 3 sub-phenotypes. Metabolic studies frequently revealed insulin resistance and hypertriglyceridemia in patients with generalized and partial LD. Regional fat loss from the thighs, with relative sparing of fat loss from the medial thighs, was more frequent in generalized than in partial LD and absent from DM patients without LD. Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in our patients. LD is an under-recognized sequela of JDM, and certain DM patients with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development of this complication. High-risk JDM patients should be screened for metabolic abnormalities, which are common in generalized and partial LD and result in much of the LD-associated morbidity. Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM.

Author List

Bingham A, Mamyrova G, Rother KI, Oral E, Cochran E, Premkumar A, Kleiner D, James-Newton L, Targoff IN, Pandey JP, Carrick DM, Sebring N, O'Hanlon TP, Ruiz-Hidalgo M, Turner M, Gordon LB, Laborda J, Bauer SR, Blackshear PJ, Imundo L, Miller FW, Rider LG, Childhood Myositis Heterogeneity Study Group

Author

Judyann C. Olson MD Associate Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Acanthosis Nigricans
Adolescent
Adult
Autoantibodies
Biomarkers
Body Fat Distribution
Calcinosis
Case-Control Studies
Child
Contracture
Dermatomyositis
Exanthema
Facial Dermatoses
Fatty Liver
Female
Follow-Up Studies
Forecasting
Hirsutism
Humans
Hypertriglyceridemia
Insulin Resistance
Lipodystrophy
Male
Muscular Atrophy
Panniculitis
Phenotype
Severity of Illness Index
Time Factors
jenkins-FCD Prod-480 9a4deaf152b0b06dd18151814fff2e18f6c05280