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

Autor: Mayuko Ishiwari, Yuki Togashi, Hiroyuki Takoi, Ryota Kikuchi, Junichiro Kawagoe, Kazutoshi Toriyama, Akane Tanaka, Yoko Nagotomo, Hayato Kinoshita, Yuta Kono, Shinji Abe
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Respiratory Medicine Case Reports, Vol 33, Iss , Pp 101464- (2021)
Druh dokumentu: article
ISSN: 2213-0071
DOI: 10.1016/j.rmcr.2021.101464
Popis: 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.
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