Early plasma exchange combined with triple-combination therapy may improve survival in patients with high mortality risk anti-MDA5-positive dermatomyositis-associated interstitial lung disease: A single-center retrospective observational study
Takuma Tsuzuki Wada, Kazuhiro Yokota, Kyohei Emoto, Shun Shinomiya, Makoto Nagata, Yuji Akiyama, Hisashi Noma, Toshihide MimuraObjective
Anti-MDA5 antibody-positive dermatomyositis (MDA5-DM) frequently causes rapidly progressive interstitial lung disease (ILD) with poor prognosis. Although immunosuppressive therapies have advanced, outcomes remain unsatisfactory. Early plasma exchange (PE) has been suggested to improve survival by removing cytokines and autoantibodies. However, patient selection for PE and its impact on survival need further exploration. This study aims to evaluate the clinical characteristics, mortality risk factors, and survival benefits of early PE combined with triple therapy in high-risk patients with MDA5-DM-ILD.
Methods
This single-center retrospective cohort study with historical controls analyzed 20 patients with MDA5-DM-ILD treated at our hospital. In Phase 1, mortality risk factors were identified by comparing eight survivors with eight non-survivors. Phase 2 evaluated the survival outcomes of four high-risk patients who underwent early PE in combination with triple therapy, and compared them with the mortality group from Phase 1 that received conventional therapy.
Results
In Phase 1, age ≥60, elevated serum CRP (≥0.8 mg/dL), and CK (≥153 U/L) were identified as factors associated with mortality in an exploratory univariate analysis. In Phase 2, all four high-risk patients who received early PE combined with triple therapy survived beyond 90 days (4/4, 100%; exact 95% confidence interval [CI], 39.8%–100%). These observations should be interpreted descriptively because of the extremely small sample size and the non-randomized historical design. However, all patients who underwent PE subsequently developed cytomegalovirus (CMV) antigenemia (asymptomatic viremia) requiring preemptive antiviral therapy.
Conclusion
Early PE intervention combined with triple therapy may be associated with favorable short-term outcomes in selected high-risk MDA5-DM-ILD patients. However, careful monitoring is required due to the potential risk of CMV reactivation. Identifying risk factors such as age, CRP, and CK levels may contribute to future risk assessment strategies, although these findings should be considered exploratory and require validation in larger multicenter studies.