Anti-MDA5 antibody-positive dermatomyositis amyopathic with rapidly progressive interstitial lung disease: a case report

Abstract

Dermatomyositis with antibodies against melanoma differentiation-associated protein 5 (DM-MDA5) is a subtype of dermatomyositis with a poor prognosis that typically presents as cutaneous manifestations and rapidly progressive interstitial lung disease (RPILD). The most prominent clinical and laboratory features of MDA5-DM are distinctive skin rashes, rapidly progressive interstitial lung disease, peripheral lymphopenia, and elevated serum ferritin levels. Concomitant infection is a frequent complication of MDA5-DM. Adequate evaluation of patients requires knowledge of the heterogeneity of the disease and variability of the clinical course. Male patients with MDA5-DM have an increased risk of RPILD elevated mortality rates, and reduced overall survival time compared to their female counterparts, and anti-Ro52 positivity may be an unfavorable prognostic factor for these patients. We report a female patient with ulcer-like skin lesions and rapidly progressive interstitial lung disease without respiratory symptoms and without muscle weakness or elevated muscle enzymes with positive MDA5 antibody, anti Ro52 positive, and elevated ferritin. The patient was treated with corticosteroids, mycophenolate mofetil and rituximab to achieve clinical remission.