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Original Research: INTERSTITIAL LUNG DISEASE |

Clinical Differences Between Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis and Classic Dermatomyositis

Hiroshi Mukae, MD, PhD; Hiroshi Ishimoto, MD; Noriho Sakamoto, MD; Shintaro Hara, MD; Tomoyuki Kakugawa, MD; Seiko Nakayama, MD; Yuji Ishimatsu, MD; Atsushi Kawakami, MD; Katsumi Eguchi, MD; Shigeru Kohno, MD, FCCP
Author and Funding Information

Affiliations: From the Second Department of Internal Medicine (Drs. Mukae, Ishimoto, Sakamoto, Hara, Kakugawa, Nakayama, Ishimatsu, and Kohno), the First Department of Internal Medicine (Drs. Kawakami and Eguchi), and the Department of General Medicine (Dr. Nakayama), Nagasaki University School of Medicine, Nagasaki, Japan.

Correspondence to: Hiroshi Mukae, MD, PhD, Second Department of Internal Medicine, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan; e-mail: hmukae@nagasaki-u.ac.jp


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(5):1341-1347. doi:10.1378/chest.08-2740
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Background:  Interstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM) is a potentially fatal condition in which the clinical features are not well understood. The aim of the present study was to clarify the differences in clinical characteristics and prognosis of patients with ILD associated with CADM (CADM-ILD) and classic dermatomyositis associated with ILD (DM-ILD).

Methods:  We retrospectively studied consecutive patients with CADM-ILD and classic DM-ILD who were hospitalized between 2001 and 2007 at Nagasaki University Hospital. The study group consisted of 11 patients with CADM-ILD and 16 patients with classic DM-ILD. We compared the clinical features and prognosis between the two forms.

Results:  The Pao2/Fio2 ratio was significantly lower in patients with CADM-ILD than in patients with classic DM-ILD. The lymphocyte subsets ratio in the BAL fluid of patients with CADM-ILD was significantly higher than the corresponding ratio in patients with classic DM-ILD. ILD is classified as acute or chronic, and the acute subtype was more common in patients with CADM-ILD than in those with classic DM-ILD. The mortality rate for patients with CADM-ILD (45%) was much higher than that for patients with classic DM-ILD (6%), and all of the CADM deaths occurred in the group of patients with acute CADM-ILD.

Conclusion:  Our data suggest that the higher prevalence of the acute subtype of ILD in patients with CADM results in a higher mortality rate for patients with CADM-ILD.

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