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 Dermatomyositis

被引：56

作者：

Teruya, Aoi ^{[1
]}

Kawamura, Kodai ^{[1
]}

Ichikado, Kazuya ^{[1
]}

Sato, Shinji ^{[2
]}

Yasuda, Yuko ^{[1
]}

Yoshioka, Masakazu ^{[1
]}

机构：

[1] Saiseikai Kumamoto Hosp, Div Resp Med, Kumamoto 8614193, Japan

[2] Tokai Univ, Sch Med, Dept Internal Med, Div Rheumatol, Isehara, Kanagawa 25911, Japan

来源：

CHEST | 2013年 / 144卷 / 06期

关键词：

GENE; 5; ANTIBODY; JAPANESE PATIENTS; AUTOANTIBODIES;

D O I：

10.1378/chest.13-0186

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

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.

引用

页码：1934 / 1936

页数：3

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