An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

Yuki Sato; Kojiro Otsuka; Koji Tamai; Yuichiro Ono; Yasuhito Hamaguchi; Keisuke Tomii

doi:10.2169/internalmedicine.56.6856

CASE REPORTS

An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

Yuki Sato, Kojiro Otsuka, Koji Tamai, Yuichiro Ono, Yasuhito Hamaguchi, Keisuke Tomii

Author information

Keywords: anti-MDA5 antibody, clinically amyopathic dermatomyositis (CADM), rapidly progressive interstitial lung disease (RP-ILD)

JOURNAL OPEN ACCESS

2017 Volume 56 Issue 3 Pages 341-346

DOI https://doi.org/10.2169/internalmedicine.56.6856

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Abstract

Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. These findings indicate that the ILD in anti-MDA5 antibody-positive patients may not only be rapidly progressive, but may also be chronic and recurrent.

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