Department of Rheumatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, China.
J Int Med Res. 2023 Sep;51(9):3000605231198742. doi: 10.1177/03000605231198742.
The prognosis of anti-MDA5-positive dermatomyositis (DM)-associated rapidly progressive interstitial lung disease (RPILD) is extremely poor and effective treatment options are limited. In addition, the risk of infection during immunosuppressive treatment is a major challenge. We report here, a case of RPILD in a 31-year-old man with anti-MDA5 antibody-positive DM. Despite treatment with methylprednisolone and human immunoglobulin, his lung condition worsened and his serum ferritin levels increased. Six cycles of plasma exchange (PE) adjuvant treatment significantly mitigated his symptoms and he was discharged from hospital two months later. We suggest that PE may be a promising therapeutic option for patients with anti-MDA5-positive DM-associated RPILD. However, randomized, controlled studies are required to confirm our findings.
抗 MDA5 阳性皮肌炎（DM）相关快速进展性间质性肺病（RPILD）的预后极差，有效的治疗选择有限。此外，免疫抑制治疗期间感染的风险是一个主要挑战。我们在此报告一例抗 MDA5 抗体阳性 DM 合并 RPILD 的 31 岁男性患者。尽管接受了甲泼尼龙和人免疫球蛋白治疗，但他的肺部情况仍恶化，血清铁蛋白水平升高。6 个周期的血浆置换（PE）辅助治疗显著缓解了他的症状，两个月后他出院。我们认为，PE 可能是抗 MDA5 阳性 DM 相关 RPILD 患者有前途的治疗选择。但是，需要进行随机对照研究来证实我们的发现。
