川口　鎮司
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Clinical Professor
Article types Original article
Language English
Peer review Non peer reviewed
Title Recent Treatment of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies.
Journal Formal name：Clinical medicine insights. Circulatory, respiratory and pulmonary medicine
Abbreviation：Clin Med Insights Circ Respir Pulm Med
ISSN code：(1179-5484)1179-5484(Linking)
Domestic / ForeginForegin
Volume, Issue, Page 9(Suppl 1),pp.9-17
Author and coauthor Kawasumi Hidenaga†, Gono Takahisa*, Kawaguchi Yasushi, Yamanaka Hisashi
Publication date 2015
Summary Interstitial lung disease (ILD) is a prognostic factor for poor outcome in polymyositis (PM)/dermatomyositis (DM). The appropriate management of ILD is very important to improve the prognosis of patients with PM/DM. ILD activity and severity depend on the disease subtype. Therefore, clinicians should determine therapeutic strategies according to the disease subtype in each patient with PM/DM. Anti-melanoma differentiation-associated gene 5 antibody and hyperferritinemia predict the development and severity of rapidly progressive (RP) ILD, particularly in East Asian patients. Combination therapy with corticosteroids, intravenous cyclophosphamide pulse, and calcineurin inhibitors should be administered in RP-ILD. In contrast, patients with anti-aminoacyl-tRNA synthetase (ARS) show better responses to corticosteroids alone. However, ILDs with anti-ARS often display disease recurrence or become refractory to corticosteroid monotherapy. Recent studies have demonstrated that the administration of tacrolimus or rituximab in addition to corticosteroids may be considered in ILD patients with anti-ARS. Large-scale, multicenter randomized clinical trials should be conducted in the future to confirm that the aforementioned agents exhibit efficacy in ILD patients with PM/DM. The pathophysiology of ILD with PM/DM should also be elucidated in greater detail to develop effective therapeutic strategies for patients with ILD in PM/DM.
DOI 10.4137/CCRPM.S23313
PMID 26279636