Early intervention of plasma exchange combined with intensive immunosuppressive treatment for anti-MDA-5 antibody–positive rapidly progressive interstitial pneumonia: Two case reports

Abstract

Background Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) has to be reported to often cause rapidly progressive interstitial lung disease (RP-ILD) especially in East Asian countries. Even with the recommended rapid administration of immunosuppressive agents with high-dose corticosteroids, intravenous pulse cyclophosphamide, and calcineurin inhibitors, the prognosis of anti-MDA5 Ab–related RP-ILD is poor. Plasma exchange (PE) has been reported to be effective for steroid-refractory RP-ILD with anti-MDA5 Ab. However, the timing, frequency, and interval of PE for the treatment of RP-ILD with anti-MDA5 Ab have not yet been established. Case presentation We report two cases of RP-ILD with anti-MDA5 Ab treated by early intervention of PE combined with immunosuppressive treatment. Blood biomarkers including titers of anti-MDA5 Ab, serum KL-6 and ferritin were promptly decreased after each session of PE. Clinical symptoms, oxygenation and chest computed tomography abnormalities were completely improved after immunosuppressive treatment with PE. Conclusion Early intervention of PE combined with immunosuppressive treatment may prevent the development to lethal severe respiratory failure in RP-ILD with anti-MDA5 Ab.

Early Initiation of Plasma Exchange Therapy for Anti-MDA5⁺Dermatomyositis with Refractory Rapidly Progressive Interstitial Lung Disease

“…Although triple immunosuppressive up-front therapy has improved its prognosis compared to conventional step-up treatment ( 5 ), patients who do not respond to such an intensive therapy at an early stage are seen in daily practice ( 1 - 5 ). Recently, case reports or case series have reported the effectiveness of PE therapy for this condition ( 11 - 15 ). In the present study, we demonstrated the effectiveness of the early initiation of PE therapy in addition to triple combination therapy.…”

“…However, RP-ILD often fails to respond to intensive treatment regimens and has a very poor prognosis (1)(2)(3)(4)(5). Therefore, other alternative treatments, including mycophenolate mofetil (6), Janus Kinase inhibitors (7,8), rituximab (9), intravenous immunoglobulin, polymyxin B immobilized fiber column direct hemoperfusion (10), and plasma exchange (PE), have been added to or replaced intensive immunosuppressive treatments, and their effectiveness has been reported in case series or case reports (11)(12)(13)(14)(15). However, no definite effects of these treatments have been reported.…”