Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) resulting in high mortality. Here we report a 51-year-old Japanese woman with anti-MDA5 antibody-positive hypomyopathic dermatomyositis (DM) who developed RP-ILD. She developed respiratory failure and pneumomediastinum, however her RP-ILD responded favorably to the combined immunosuppressive treatments consisting of steroids, intravenous cyclophosphamide and tacrolimus. She was complicated with severe infections, which were successfully managed by combined modality therapy including artificial ventilation and antibiotics in addition to immunosuppressive treatments in parallel to the decline of anti-MDA5 antibody titer (>150 Index to 75 Index). She was discharged after 6 months of treatment without any respiratory sequelae. Hypomyopathic DM patients with high titers of anti-MDA5 antibody should be treated with aggressive immunosuppressive therapies and closely monitored to prevent various infections.
Keywords: Anti-MDA5 antibody; dermatomyositis; interstitial lung disease; pneumomediastinum.