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: |
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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. |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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