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Abstract Number: 0684

Extended Report: Successful Treatment anti-MDA5 Antibody-positive Interstitial Lung Disease with Plasma Exchange Therapy

Yoshiyuki Abe1, Masahiro Kogami2, Makio Kusaoi2, Kurisu Tada3, Ken yamaji4 and Naoto Tamura3, 1Juntendo University School of Medicine, Tokyo, Japan, 2Juntendo University, Tokyo, Japan, 3Department of Internal Medicine and Rheumatology, Juntendo University Faculty of Medicine, Tokyo, Japan, 4Juntendo University School of Medicine Internal Medicine and Rheumatology, Tokyo, Japan

Meeting: ACR Convergence 2021

Keywords: dermatomyositis, interstitial lung disease, Mortality, Myositis

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Session Information

Date: Sunday, November 7, 2021

Session Title: Muscle Biology, Myositis & Myopathies Poster (0683–0722)

Session Type: Poster Session B

Session Time: 8:30AM-10:30AM

Background/Purpose: This study was extended report from our previous study that “Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy” (Rheumatology (Oxford) 59(4):767-71, 2020). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are a type of myositis-specific autoantibody. Anti-MDA5 antibodies are closely related to interstitial lung disease (ILD). Intensive immunosuppressive therapy with combination calcineurin inhibitor, and intravenous pulse cyclophosphamide was developed, because rapid progressive-ILD (RP-ILD) with anti-MDA5 antibodies is refractory and fatal. Intensive immunosuppressive therapy was shown to improve patient survival and prognosis. However, 20–30% of cases were still fatal, and we reported that the effectiveness of plasma exchange (PE) therapy for refractory RP-ILD. We aimed to reveal the effect of PE on survival in patients with refractory RP-ILD with anti-MDA5 antibodies.

Methods: We added 6 RP-ILD patients who were positive of anti-MDA5 antibodies with PE. Refractory RP-ILD was defined as radiological progression and/or oxygenation exacerbation within 4 weeks after intensive immunosuppressive therapy. Study approval was obtained from the ethical committee of Juntendo University Hospital (approval number 17-274).

Results: The PE group included 6 former patients and 6 new patients such as 1 with newly refractory cases, 3 with relapsed refractory cases, and 2 with newly severe hypoxia cases that P/F ratio was less than 200. A total of 12 patients were included in the PE group. The 1-year survival rate of the PE group was significantly higher than that of the non-PE group (92% and 25%, respectively, P = 0.03). One patient who showed severe hypoxia at the start of treatment was improved from respiratory failure, but he died of candidemia due to vascular catheter infection.

Conclusion: We revealed the higher 1-year survival rate of PE for refractory or severe hypoxia RP-ILD in patients positive for anti-MDA5 antibodies. Physicians may consider that adding PE in refractory or severe hypoxia RP-ILD patients positive for anti-MDA5 antibodies.

Kaplan-Meier curves for survivability comparing the overall PE group and non-PE group.
The result of the log-rank test was P = 0.005. PE: plasma exchange therapy.


Disclosures: Y. Abe, GlaxoSmithKline, 5; M. Kogami, None; M. Kusaoi, None; K. Tada, None; K. yamaji, None; N. Tamura, AbbVie Japan GK, 6, Bristol-Myers Squibb Co. Ltd, 6, Chugai Pharmaceutical Co. Ltd, 6, Eisai Co. Ltd, 6, Eli Lilly Japan K.K, 6, Glaxo Smith Kline K.K., 6, Janssen Pharmaceutical K.K., 6, Mitsubishi-Tanabe Pharma Co., 6, Novartis Pharma K.K, 6.

To cite this abstract in AMA style:

Abe Y, Kogami M, Kusaoi M, Tada K, yamaji K, Tamura N. Extended Report: Successful Treatment anti-MDA5 Antibody-positive Interstitial Lung Disease with Plasma Exchange Therapy [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/extended-report-successful-treatment-anti-mda5-antibody-positive-interstitial-lung-disease-with-plasma-exchange-therapy/. Accessed January 27, 2023.
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