Long-term outcome of juvenile dermatomyositis associated with lipodystrophy: experience of a university hospital

TL;DR

This study examines the long-term outcomes of children with juvenile dermatomyositis who developed lipodystrophy, finding that delayed diagnosis and treatment are linked to severe complications.

Contribution

The paper highlights lipodystrophy as a rare but severe complication of juvenile dermatomyositis and emphasizes the importance of early diagnosis and treatment.

Findings

Five out of 22 JDM patients (23%) developed lipodystrophy, with no metabolic complications observed.

Delayed diagnosis and standard immunosuppressive therapy were associated with progression to generalized lipodystrophy and calcinosis.

Three patients had chronic JDM requiring ongoing treatment, while two achieved remission with residual lipodystrophy.

Abstract

Juvenile Dermatomyositis (JDM) is a rare myopathy. Lipodystrophy is an under-reported chronic JDM complication.

Assess the long-term outcome of JDM associated with lipodystrophy.

Retrospective study of JDM patients who developed lipodystrophy, diagnosed and treated between 1st January 1990 and 31st December 2023, in a University Clinic of Dermatology. The mean follow-up was 150.8-months (range 29‒291).

Lipodystrophy was diagnosed in 5 children (4 girls and one boy, aged 3‒14 years) of 22 JDM patients (17 girls and 5 boys). Four patients had partial, and one had focal lipodystrophy that occurred 34.2-months (mean period) after the initial JDM symptoms. All five children had antinuclear antibodies, but none had dermatomyositis-specific/associated antibodies. No malignancies or visceral involvement were found. At JDM presentation, all 5 patients had low serum vitamin D, body mass index (BMI) ranged from 14.3 to 20.7. Triglycerides and fasting glucose levels were normal in all patients, while cholesterol was elevated in one patient. Despite Standard Immunosuppressive Therapy (IST), 2/4 of patients with initially partial lipodystrophy progressed to generalized lipodystrophy and 4/5 patients developed calcinosis 46 months (mean period) after JDM diagnosis. At the last check-up, 3/5 patients had chronic JDM course, requiring IST, while 2/5 patients were in remission, without IST, with only residual lipodystrophy.

Retrospective study.

During the 34-year period, 5/22 (23%) JDM patients developed lipodystrophy. Metabolic complications were not found. All patients had significant delays in JDM diagnosis. Early diagnosis and IST are necessary to reduce the risk of lipodystrophy, as a severe, chronic JDM complication.

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