Acute exacerbations of interstitial lung diseases

Curr Opin Pulm Med. 2010 Sep;16(5):480-6. doi: 10.1097/MCP.0b013e32833ae49d.


Purpose of review: This review aims to highlight recent advances in pathogenesis, clinical presentation and treatment of interstitial lung diseases (ILDs).

Recent findings: Acute exacerbation is increasingly recognized as a major complication in the course of idiopathic pulmonary fibrosis. It is precipitated by a variety of intrinsic and extrinsic factors. Moreover, acute exacerbation is an apparently equally frequent event in hypersensitivity pneumonitis and collagen-vascular diseases associated ILDs, especially the rheumatoid pulmonary fibrosis. Treatment of acute exacerbations is unsatisfactory and prognosis extremely poor.

Summary: In a critically ill patient proper recognition of an acute exacerbation and of the underlying chronic ILD is warranted because treatment approach varies with the type of ILD. Advances in the understanding of the pathogenesis and treatment of this 'idiopathic' phenomenon are reviewed.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Collagen Diseases / complications
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / physiopathology*
  • Pulmonary Fibrosis / complications
  • Sarcoidosis, Pulmonary / complications
  • Vascular Diseases / complications


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents