One-year survival benefit of plasma exchange in idiopathic inflammatory myositis patients with progressive interstitial lung disease-a systemic review and meta-analysis

Abstract

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. All the statistical analyses were performed using RevMan version 4.12.0.

Results

Out of 438 retrieved studies, 16 were selected for full-text review. Six cohort studies involving 148 patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis or antisynthetase syndrome-related dermatomyositis with rapidly progressive ILD refractory to standard treatments (including glucocorticoids, immunosuppressive agents, or intravenous immunoglobulin) met the inclusion criteria. Patients receiving PLEX in addition to other therapies demonstrated a greater one-year survival rate (relative risk [RR] 1.59, 95 % CI 0.96–2.65, I² 52 %) than did patients in the non-PLEX group. Significance was reached in a sensitivity analysis after excluding one outlier (RR 1.71, 95 % confidence intervals [CI] 1.30–2.25; I² 0 %). Additionally, there was a trend suggesting that PLEX improved lung function, radiographic outcomes, and key serum biomarkers, such as Krebs von den Lungen-6 and ferritin. Funnel plot asymmetry suggested publication bias due to the lack of reporting of negative trials. All studies had a low risk of bias.

Conclusions

As an adjunctive therapy, PLEX improved one-year survival in IIM patients with rapidly progressive ILD who were unresponsive to standard treatments.