Updated guidelines on the treatment of idiopathic pulmonary fibrosis (IPF) have been released by an international group of  leading respiratory societies. The new guidelines, issued by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association, were published in the American Thoracic Society’s American Journal of Respiratory and Critical Care Medicine.

“In these updated guidelines, we analyzed new evidence reported since our 2011 guideline was issued and updated our treatment recommendations accordingly,” said Ganesh Raghu, MD, Professor of Medicine, University of Washington, director of the Center for Interstitial Lung Disease, UW Medicine at the University of Washington Medical Center, and chair  of the committee that produced the guidelines.

“The updated guidelines do not recommend one treatment regimen over another. All of these recommendations must be weighed individually, considering all the factors used to grade each one, including the confidence in effect estimates, evidence from outcomes studies, desirable and undesirable consequences of treatment, treatment costs, the implications of treatment on health equity, and the feasibility of treatment,” Raghu added.

Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach, with recommendations rated as either “strong” or “conditional.” Conditional recommendations are synonymous with weak recommendations.

The following recommendations are new or revised from the 2011 guidelines:

  • The recommendation against the use of the following agents for the treatment of IPF is strong:
    • Anticoagulation (warfarin)
    • Imatinib, a selective tyrosine kinase inhibitor against platelet-derived growth factor (PDGF) receptors
    • Combination prednisone, azathioprine, and N-acetylcysteine
    • Selective endothelin receptor antagonist (ambrisentan)
  • The recommendation for the use of the following agents for the treatment of IPF is conditional:
    • Nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors
  • The recommendation against the use of the following agents for the treatment of IPF is conditional:
    • Phosphodiesterase-5 inhibitor (sildenafil)
    • Dual endothelin receptor antagonists (macitentan, bosentan)
  • Recommendations remaining unchanged from the 2011 guidelines include a conditional recommendation against the use of N-acetylcysteine monotherapy for IPF and a conditional recommendation for the use of antiacid therapy.

“Our systematic review of the available evidence on IPF treatments points to the need for additional research and long-term studies of their safety and efficacy,” said Raghu. “This is especially true for treatments that received conditional recommendations in the guidelines. The guidelines empower the clinician to make the most appropriate treatment choices for the patient confronted with IPF and encourage shared decision-making with the well informed patient to choose the most appropriate treatment options tailored to the individual patient’s needs.”