Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis

Abstract

A 73-year-old woman who visited our hospital complaining of dry cough for three months was refractory to antimicrobial therapy. Chest computed tomography revealed subpleural consolidation. Specimens obtained from surgical lung biopsy revealed subpleural perilobular airspace organization and fibrosis. After the biopsy, mechanic's hand and Gottron's papules appeared, and anti-melanoma differentiation-associated gene 5 (MDA5) antibody was found to be positive. Subsequently, anti-MDA5 antibody measured in cryopreserved serum from her first admission proved to be positive. It is difficult to suspect the presence of anti-MDA-5 antibody in patients with interstitial lung disease without typical dermatomyositis symptoms or slow disease progression.

Asymptomatic and slowly progressive anti-MDA5 ILD: A report of three cases deviating from a notoriously rapidly progressive ILD

“…Various scoring systems have been proposed to identify indicators for development of RP-ILD in anti-MDA5 patients in cohorts of patients with acute and chronic ILD [ 4 , 5 ]. However, asymptomatic or slowly progressive cases of anti-MDA5 ILD are not well described in the literature [ 6 ]. We report three cases of Latino patients with asymptomatic or slowly progressive anti-MDA5 ILD ( Table 1 , Fig.…”