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Clinical Rheumatology
Published in: Clinical Rheumatology 10/2007

01-10-2007 | Original Article

Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study

Authors: Shuang Ye, Xiao-xiang Chen, Xiao-ye Lu, Mei-fang Wu, Yun Deng, Wen-qun Huang, Qiang Guo, Cheng-de Yang, Yue-ying Gu, Chun-de Bao, Shun-le Chen

Published in: Clinical Rheumatology | Issue 10/2007

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Abstract

The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer’s definition or Bohan–Peter’s classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan–Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR = 4.237, CI 95%: 1.239–14.49, p = 0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO2, higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation.
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Metadata
Title
Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study
Authors
Shuang Ye
Xiao-xiang Chen
Xiao-ye Lu
Mei-fang Wu
Yun Deng
Wen-qun Huang
Qiang Guo
Cheng-de Yang
Yue-ying Gu
Chun-de Bao
Shun-le Chen
Publication date
01-10-2007
Publisher
Springer-Verlag
Published in
Clinical Rheumatology / Issue 10/2007
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-007-0562-9

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