Idiopathic Pulmonary Fibrosis (IPF) Guidelines

Updated: Jul 15, 2019
  • Author: Amanda M K Godfrey, MD; Chief Editor: Guy W Soo Hoo, MD, MPH  more...
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Guidelines

Guidelines Summary

The 2015 guidelines on idiopathic pulmonary fibrosis (IPF) by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association include the following points [52, 53] :

  • The guidelines recommend against the use of the following agents for the treatment of idiopathic pulmonary fibrosis: anticoagulation (warfarin); imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor (PDGF) receptors; combination prednisone, azathioprine, and N-acetylcysteine; and selective endothelin receptor antagonist (ambrisentan).
  • The guidelines also give a conditional recommendation against the use of sildenafil, macitentan, and bosentan.
  • The panel has continued the 2011 conditional recommendation against the use of N-acetylcysteine monotherapy for IPF. They have also continued the 2011 conditional recommendation for the use of antacid therapy in patients without symptoms of GER.
  • The recommendation for the use of the following agents for the treatment of idiopathic pulmonary fibrosis is conditional: nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors; and pirfenidone.

The clinical practice guidelines on the diagnosis of idiopathic pulmonary fibrosis were released on September 1, 2018, also by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. [3, 54] The collaborative guidelines outline basic recommendations for clinical observation, HRCT scanning, bronchoscopy, histopathology, and serum biomarker measurements, as follows:

  • Obtain a detailed history for possible environmental exposures or medication use to exclude potential causes of interstitial lung disease (ILD).
  • Perform serologic testing to exclude connective-tissue disease.
  • With newly diagnosed ILD, perform HRCT scanning of the chest; determine if findings indicate a pattern of (1) usual interstitial pneumonia (UIP), (2) probable UIP, (3) indeterminate of UIP, or (4) alternate diagnosis.
  • For probable UIP, indeterminate findings, or alternate diagnosis, bronchoalveolar lavage and surgical lung biopsy are recommended; evidence is insufficient to recommend for or against transbronchial lung biopsy or lung cryobiopsy.
  • For a UIP pattern, bronchoalveolar lavage (cellular analysis) and surgical lung biopsy are not recommended (risks outweigh benefits); guidelines strongly recommend against transbronchial lung biopsy or lung cryobiopsy in this situation.
  • Multidisciplinary discussions (MDDs) should include interactions between pulmonologists, possibly rheumatologists (on a case-by-case basis), pathologists, and radiologists. MDDs are recommended with newly detected ILD of an unknown cause in patients whose findings clinically suggest idiopathic pulmonary fibrosis. MDDs likely provide the greatest benefit in situations in which HRCT patterns indicate probable UIP, indeterminate findings, or alternative diagnoses, or when clinical, histologic, or radiologic data are incongruous.
  • For serum biomarker measurements, measuring serum matrix metalloproteinase 7, chemokine ligand 18, Krebs von den Lungen-6, or surfactant protein D is not recommended solely to distinguish idiopathic pulmonary fibrosis from other ILDs, owing to high false-positive and false-negative results.