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大学・研究所にある論文を検索できる 「Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High-Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis」の論文概要。リケラボ論文検索は、全国の大学リポジトリにある学位論文・教授論文を一括検索できる論文検索サービスです。

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Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High-Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti-Melanoma Differentiation-Associated Gene 5-Positive Dermatomyositis

Tsuji, Hideaki 京都大学 DOI:10.14989/doctor.k22883

2021.01.25

概要

Objective: Interstitial lung disease (ILD) accompanied by anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis (DM) is often rapidly progressive and associated with poor prognosis. Because there is no established treatment, we prospectively evaluated the efficacy and safety of a combined immunosuppressive regimen for anti-MDA5-positive DM with ILD.

Methods: Adult Japanese patients with newly onset anti-MDA5-positive DM with ILD (n = 29) were enrolled in multiple centers from 2014 to 2017. They were treated with a regimen of high-dose glucocorticoids (GC), tacrolimus, and intravenous cyclophosphamide (IVCY). Plasmapheresis was used if patients worsened after the regimen started. The primary endpoint was six-month survival, compared with that of a historical control (n = 15) of patients with anti-MDA5-positive DM with ILD who received step-up treatment (high-dose GC and stepwise addition of immunosuppressant). Secondary endpoints were 12-month survival rate, adverse events, and changes in laboratory data.

Results: The combined immunosuppressive regimen group showed significantly higher 6-month survival than the step-up treatment group (89% and 33%, respectively, P < 0.0001). Over 52 weeks, anti-MDA5 titer, serum ferritin level, percent vital capacity, and chest high-resolution computed tomography scores improved. The combined immunosuppressive regimen group received IVCY nearly 20 days earlier with shorter intervals and tended to receive plasmapheresis more often than patients undergoing step-up treatment (n = 15). Cytomegalovirus reactivation was frequently observed over 52 weeks.

Conclusion: A combined immunosuppressive regimen is effective for anti-MDA5-positive DM with ILD. Plasmapheresis can be used for additional effect in intractable disease. Opportunistic infections should be carefully monitored during treatment.

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