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.
The result of the log-rank test was P = 0.005. PE: plasma exchange therapy.
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.« Back to ACR Convergence 2021
ACR Meeting Abstracts - https://acrabstracts.org/abstract/extended-report-successful-treatment-anti-mda5-antibody-positive-interstitial-lung-disease-with-plasma-exchange-therapy/