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

doi:10.1378/chest.08-2740

Chest

Volume 136, Issue 5, November 2009, Pages 1341-1347

https://doi.org/10.1378/chest.08-2740 Get rights and content

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.

Section snippets

Patient and Diagnostic Criteria

We retrospectively studied 27 consecutive Japanese patients with CADM and classic DM-ILD who were hospitalized at the Nagasaki University Hospital from 2001 to 2007. This hospital is a referral center in this area for ILD. All patients were referred to our hospital for the evaluation of dermatomyositis-associated ILD. Some patients were admitted to the hospital for diagnostic evaluation in the absence of clinical deterioration, whereas others were admitted for the evaluation of respiratory

Clinical Features and Laboratory Findings

Eleven patients (two men and nine women; mean [± SD] age, 60.4 ± 10.7 years; current smokers, two patients; never-smokers, nine patients) were given a diagnosis of CADM-ILD, and 16 patients (one man and 15 women; mean age, 53.3 ± 11.1 years; never-smokers, 16 patients) were given a diagnosis of classic DM-ILD. There were no statistically significant differences in the male/female ratio and the age distribution between CADM and classic dermatomyositis. No patients had any malignancies or

Discussion

CADM is a rare form of dermatomyositis that typically manifests with characteristic cutaneous lesions and mild or no muscle involvement.5, 13 Cottin et al¹⁹ reported a favorable prognosis for patients with CADM-ILD, but there have been reports,6, 7, 8, 9, 10 mainly from Asian regions, of several cases of fatal ILD associated with CADM. These observations suggest that CADM includes heterogeneous disease populations. The present study showed that the mortality rate of patients with CADM-ILD was

Acknowledgments

Author contributions: Dr. Mukae designed the article, acquired the data, analyzed and interpreted the data, and drafted the article. Drs. Ishimoto, Sakamoto, Hara, Kakugawa, Nakayama, Ishimatsu, and Kawakami acquired the data, interpreted the data, and helped draft the article. Drs. Eguchi and Kohno interpreted the data and helped draft the article.

Financial/nonfinancial disclosures: The authors have reported to the ACCP that no significant conflicts of interest exist with any

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