Successful treatment of rapidly progressive unclassifiable idiopathic interstitial pneumonia with anti-melanoma differentiation-associated gene-5 antibody by intensive immunosuppressive therapy

Abstract

We describe a case of a woman who presented with a persistent cough, general fatigue, and a fever. Interstitial lung disease was rapidly progressive and resistant to high-dose steroid therapy. She tested positive for the presence of anti-melanoma differentiation-associated gene 5 (MDA-5) antibody, although she had no skin manifestations of dermatomyositis. She was eventually diagnosed with unclassifiable idiopathic interstitial pneumonia and was successfully treated with intensive immunosuppressive therapy including intravenous cy-clophosphamide. To our knowledge, this is the first report of anti-MDA-5 antibody in a patient with idiopathic interstitial pneumonia.