Objectives: This study aimed to systematically evaluate the efficacy of plasma exchange (PLEX) in patients with idiopathic inflammatory myositis (IIM) complicated by interstitial lung disease (ILD).
Method: We conducted a comprehensive literature search in Medline and EMBASE from their inception to August 2023, focusing on randomized controlled trials, cohort studies, and case-control studies involving IIM patients with ILD treated with PLEX compared to those treated with standard therapies. The primary outcome was the one-year survival rate.