Interstitial lung disease in the connective tissue diseases

Clin Chest Med. 2012 Mar;33(1):123-49. doi: 10.1016/j.ccm.2012.01.004.

Abstract

The connective tissue diseases (CTDs) are inflammatory, immune-mediated disorders in which interstitial lung disease (ILD) is common and clinically important. Interstitial lung disease may be the first manifestation of a CTD in a previously healthy patient. CTD-associated ILD frequently presents with the gradual onset of cough and dyspnea, although rarely may present with fulminant respiratory failure. Infection and drug reaction should always be ruled out. A diagnosis of idiopathic ILD should never be made without a careful search for subtle evidence of underlying CTD. Treatment of CTD-ILD typically includes corticosteroids and immunosuppressive agents.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Autoantibodies / blood*
  • Bronchoalveolar Lavage
  • Connective Tissue Diseases / complications*
  • Connective Tissue Diseases / diagnosis*
  • Connective Tissue Diseases / drug therapy
  • Connective Tissue Diseases / immunology
  • Cough / etiology
  • Disease Progression
  • Dyspnea / etiology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Lung / diagnostic imaging
  • Lung / pathology*
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / pathology
  • Pulmonary Fibrosis / diagnosis
  • Pulmonary Fibrosis / etiology
  • Radiography
  • Respiratory Function Tests
  • Respiratory Insufficiency / etiology

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Immunosuppressive Agents