Idiopathic Pulmonary Fibrosis Guidelines

Updated: Aug 11, 2016
  • Author: Amanda M K Godfrey, MD; Chief Editor: Ryland P Byrd, Jr, MD  more...
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Guidelines

Guidelines Summary

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 [40, 41] :

  • 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.