Managing diagnostic procedures in idiopathic pulmonary fibrosis

Eur Respir Rev. 2013 Jun 1;22(128):158-62. doi: 10.1183/09059180.00001213.


Idiopathic pulmonary fibrosis (IPF), the most prevalent of the idiopathic interstitial pneumonias, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for its optimal management. The 2011 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway, with formal designation of criteria for an HRCT pattern of usual interstitial pneumonia. In the correct clinical context, a usual interstitial pneumonia pattern on HRCT is indicative of a definite diagnosis of IPF and negates the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of the guideline recommendations by clinicians has identified limitations that should be addressed in future statements. Key problems include: 1) HRCT misdiagnosis, particularly by less experienced radiologists; 2) lack of management recommendations for the highly prevalent clinical scenarios of "probable" or "possible" IPF; 3) ongoing confusion concerning the diagnostic role of bronchoalveolar lavage; and 4) the lack of integration of clinical data in the designation of the diagnostic likelihood of IPF, including the treated course of disease. These issues become evident as the recommendations are applied and highlight the need for continued guideline adjustments.

Keywords: Bronchoalveolar lavage; diagnosis; guidelines; high-resolution computed tomography; idiopathic pulmonary fibrosis; surgical lung biopsy.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Biopsy
  • Bronchoalveolar Lavage
  • Consensus
  • Diagnostic Techniques, Respiratory System* / standards
  • Humans
  • Idiopathic Pulmonary Fibrosis / diagnosis*
  • Practice Guidelines as Topic
  • Predictive Value of Tests
  • Prognosis
  • Tomography, X-Ray Computed