Abstract
Anti-MDA5-positive clinically amyopathic dermatomyositis (CADM) is closely linked to rapidly progressive-interstitial lung disease (RP-ILD) and is associated with high mortality rates. This report discusses a 60-year-old female patient diagnosed with anti-MDA5-positive CADM who developed RP-ILD that was resistant to pulse steroid therapy, tacrolimus, and intravenous immunoglobulin. As her respiratory condition worsened alongside rising inflammatory markers, therapeutic plasma exchange (TPE) was initiated. Following four sessions of TPE administered on alternate days, significant clinical and laboratory improvements were observed. This case underscores the potential life-saving impact of early TPE for patients with anti-MDA5-positive CADM and RP-ILD who do not respond to traditional immunosuppressive treatments.