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

Shuang Ye1, Xiao-xiang Chen, Xiao-ye Lu

  • 1Department of Rheumatology, Renji Hospital, Medical School of Shanghai Jiao Tong University, 145 Shandong (c) Rd, Shanghai 20001, China. yeshuang@online.sh.cn

Clinical Rheumatology
|February 20, 2007
PubMed

Insights

Clinically amyopathic dermatomyositis with interstitial lung disease (ILD) progresses rapidly and is often fatal. Early identification of ILD in dermatomyositis and polymyositis is crucial for predicting patient outcomes.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Internal Medicine

Background:

  • Dermatomyositis (DM) and polymyositis (PM) are inflammatory myopathies.
  • Clinically amyopathic dermatomyositis (CADM) is a subtype of DM characterized by minimal or absent skin manifestations.
  • Interstitial lung disease (ILD) is a serious complication of DM and PM.

Purpose of the Study:

  • To investigate the clinical characteristics of adult CADM with rapidly progressive ILD.
  • To identify predictive factors for ILD in DM/PM patients.
  • To explore the clinical course and prognostic indicators of ILD in DM/PM.

Main Methods:

  • Retrospective study of 145 hospitalized DM/PM patients (1998-2005).
  • Classification into CADM, classic DM, or PM using established criteria.
  • Stratification based on the presence or absence of clinical ILD.
  • Kaplan-Meier survival analysis and COX regression to identify risk factors and survival rates.

Main Results:

  • ILD was a significant risk factor for poor outcomes in DM/PM (OR=4.237).
  • CADM-ILD showed a rapidly progressive course with a 6-month survival rate of 40.8%.
  • DM-ILD had a 5-year survival rate of 54%, while PM-ILD was chronic with higher survival rates.
  • Preserved muscle strength, elevated ESR, and hypoalbuminemia may indicate ILD.
  • Antinuclear antibody presence appeared protective.

Conclusions:

  • ILD is a common and significant prognostic indicator in DM/PM, affecting over 40% of hospitalized patients.
  • Rapidly progressive CADM-ILD is refractory to conventional treatment and requires further investigation.
  • CADM represents a distinct phenotype of DM/PM, with CADM-ILD being particularly aggressive and often fatal.

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