Long-Term Prognosis of Antimelanoma Differentiation-Associated Gene 5-Positive Dermatomyositis With Interstitial Lung Disease.

Autor:	Sasai T; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Nakashima R; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto; ranran@kuhp.kyoto-u.ac.jp., Tsuji H; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Nakajima T; T. Nakajima, MD, PhD, Y. Imura, MD, PhD, Department of Clinical Immunology and Rheumatology, Medical Research Institute Kitano Hospital, Osaka., Imura Y; T. Nakajima, MD, PhD, Y. Imura, MD, PhD, Department of Clinical Immunology and Rheumatology, Medical Research Institute Kitano Hospital, Osaka., Yoshida Y; Y. Yoshida, MD, PhD, S. Hirata, MD, PhD, Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima., Hirata S; Y. Yoshida, MD, PhD, S. Hirata, MD, PhD, Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima., Shirakashi M; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Hiwa R; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Kitagori K; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Akizuki S; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Yoshifuji H; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto., Mimori T; T. Mimori, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, and Ijinkai Takeda General Hospital, Kyoto, Japan., Morinobu A; T. Sasai, MD, R. Nakashima, MD, PhD, H. Tsuji, MD, PhD, M. Shirakashi, MD, PhD, R. Hiwa, MD, PhD, K. Kitagori, MD, PhD, S. Akizuki, MD, PhD, H. Yoshifuji, MD, PhD, A. Morinobu, MD, PhD, Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto.
Jazyk:	angličtina
Zdroj:	The Journal of rheumatology [J Rheumatol] 2023 Nov; Vol. 50 (11), pp. 1454-1461. Date of Electronic Publication: 2023 Sep 15.
DOI:	10.3899/jrheum.2023-0371
Abstrakt:	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. (Copyright © 2023 by the Journal of Rheumatology.)
Databáze:	MEDLINE
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