Rapidly Progressive Interstitial Lung Disease Originating from Clinically Amyopathic Dermatomyositis Associated with Positive Anti-MDA 5 Antibody: a Case Report and Literature Review

Abstract

Clinically amyopathic dermatomyositis (CADM) is a rare auto-immune disease, which discriminates from classical dermatomyositis; it lacks typical muscle weakness, but has a high risk of developing rapid progressive interstitial lung disease (RP-ILD), malignancies, and other fatal complications, so it is easy to cause misdiagnosis and high mortality. To attract more attention and arouse more understanding, we cover and summarize our clinical findings. Here, we reported a male patient, who rapidly progressed to serious respiratory failure despite an aggressive treatment, a combination of methylprednisolone, cyclosporine, acetylcysteine, and intravenous immunoglobulin. He suffered from CADM with positive anti-melanoma differentiation-associated gene-5 (MDA 5) antibody. CADM, as a special phenotype of DM/PM (dermatomyositis/polymyositis), is usually rapidly progressive and fatal, which requires further investigation into specific pathogenesis and more effective diagnosis as well as treatment measures. Due to the severity and rarity, we simultaneously performed a review of recent literature to provide more theoretical references for clinicians in diagnosis and treatment and finally improve the survival rate of these patients.