Long-Term Prognosis of Antimelanoma Differentiation–Associated Gene 5–Positive Dermatomyositis With Interstitial Lung Disease
Tsuneo Sasai, Ran Nakashima, Hideaki Tsuji, Toshiki Nakajima, Yoshitaka Imura, Yusuke Yoshida, Shintaro Hirata, Mirei Shirakashi, Ryosuke Hiwa, Koji Kitagori, Shuji Akizuki, Hajime Yoshifuji, Tsuneyo Mimori, Akio Morinobu
Abstract
Objective Antimelanoma differentiation–associated gene 5 (anti-MDA5)–positive dermatomyositis with interstitial lung disease (DM-ILD) progresses rapidly and has a poor prognosis. Previously, we reported the efficacy of a combination therapy comprising high-dose glucocorticoids (GCs), calcineurin inhibitors (CNIs), and intravenous cyclophosphamide (IV CYC) in a multicenter clinical trial (UMIN000014344). In the present study, we evaluated the long-term outcomes and effects of induction therapy on the maintenance of remission. Methods All participants from our previous trial were followed up for > 5 years. Seventy-three other patients with anti-MDA5–positive DM-ILD from our institute were retrospectively integrated into the previous trial for further analysis. Sixty-eight patients achieved remission and survived for > 6 months. Based on the induction treatment, we classified the patients into 2 groups: (1) group T (n = 56), with triple combination therapy (GCs, CNIs, and IV CYC), and (2) group C (n = 12), with monotherapy/dual therapy. The recurrence-free and drug-withdrawal rates of immunosuppressive agents were compared. Results The overall survival and recurrence-free survival rates at 5 years were 100% for the participants in the previous trial. The 5-year cumulative withdrawal rates for CNIs and GCs were 70% and 53%, respectively. In a comprehensive analysis, the recurrence-free rates in group T were higher than those in group C (90% vs 56%; P < 0.05). The drug-withdrawal rates of CNIs and GCs at 10 years in group T were also higher than those in group C (79% vs 0% and 43% vs 0%, respectively; P < 0.05). Conclusion Triple combination therapy in the induction phase can reduce the risk of recurrence and facilitate drug withdrawal in anti-MDA5–positive DM-ILD.