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Are respiratory complications common causes of death in inflammatory myopathies? An autopsy study

Fabien Maldonado1, Rajesh R Patel, Vivek N Iyer

  • 1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA. maldonado.fabien@mayo.edu

Respirology (Carlton, Vic.)
|November 17, 2011
PubMed
Abstract

Insights

Pulmonary complications, infections, and cardiovascular diseases are leading causes of death in polymyositis (PM) and dermatomyositis (DM) patients. Nearly one-third of deaths were unexpected, highlighting diagnostic challenges in these inflammatory myopathies.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Pathology

Background:

  • Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies.
  • These conditions present with diverse clinical manifestations, notably pulmonary complications.

Purpose of the Study:

  • To investigate the primary causes of mortality in patients diagnosed with polymyositis or dermatomyositis.
  • To analyze the accuracy of ante-mortem clinical diagnoses compared to post-mortem findings.

Main Methods:

  • Retrospective analysis of 39 PM/DM patients who underwent autopsy at Mayo Clinic from 1981-2009.
  • Review of clinical data and autopsy findings to determine immediate and contributing causes of death.
  • Comparison of clinical diagnoses with autopsy-based diagnoses.

Main Results:

  • Respiratory diseases (33%), infections (28%), and cardiovascular diseases (26%) were the most frequent immediate causes of death.
  • Acute exacerbation of interstitial lung disease and bronchopneumonia were the most common specific causes.
  • In approximately one-third of cases, the immediate cause of death was not suspected clinically.

Conclusions:

  • Pulmonary injury is the immediate cause of death in one-third of PM/DM patients.
  • Acute exacerbation of chronic interstitial lung disease and bronchopneumonia are key fatal pulmonary complications.
  • A significant proportion of deaths had causes not identified ante-mortem, suggesting potential for improved diagnosis and treatment.

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