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Successful Polymyxin B Hemoperfusion Treatment Associated With Serial Reduction of Serum Anti-CADM-140/MDA5 Antibody Levels in Rapidly Progressive Interstitial Lung Disease With Amyopathic DermatomyositisRapidly Progressive Interstitial Lung Disease

Aoi Teruya, MD; Kodai Kawamura, MD, PhD; Kazuya Ichikado, MD, PhD; Shinji Sato, MD, PhD; Yuko Yasuda, MD; Masakazu Yoshioka, MD, PhD
Author and Funding Information

From the Division of Respiratory Medicine (Drs Teruya, Kawamura, Ichikado, Yasuda, and Yoshioka), Saiseikai Kumamoto Hospital, Chikami, Kumamoto; and the Division of Rheumatology (Dr Sato), Department of Internal Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan.

Correspondence to: Aoi Teruya, MD, Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, 5-3-1, Chikami, Kumamoto, 861-4193, Japan; e-mail: tanaka.aoi10003@gmail.com


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(6):1934-1936. doi:10.1378/chest.13-0186
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Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.

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