Guideline updates treatments for idiopathic pulmonary fibrosis
The guideline included a strong recommendation against the use of warfarin, among other medications.
An updated guideline offered some changes in standards of care for idiopathic pulmonary fibrosis (IPF).
The update from 2011 guideline was drafted by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association. Results appeared online in the American Journal of Respiratory and Critical Care Medicine on July 15. A free executive summary is also online.
The following recommendations are new or revised from the previous guideline:
- a strong recommendation against the use of warfarin (low confidence in effect estimates); imatinib (moderate confidence in effect estimates); combination prednisone, azathioprine, and N-acetylcysteine (low confidence in effect estimates); and the selective endothelin receptor antagonist ambrisentan (low confidence in effect estimates),
- a conditional recommendation for the use of nintedanib (moderate confidence in effect estimates) and pirfenidone (moderate confidence in effect estimates), and
- a conditional recommendation against the use of the phosphodiesterase-5 inhibitor sildenafil (moderate confidence in effect estimates) or the dual endothelin receptor antagonists macitentan and bosentan (low confidence in effect estimates).
Unchanged from the 2011 guideline were a conditional recommendation against N-acetylcysteine monotherapy based on low confidence in effect estimate and a conditional recommendation for antacid therapy based on very low confidence in effect estimate. The guideline authors noted that recommendations on treatment of pulmonary hypertension associated with IPF were deferred until the next update and that analysis of treatment of acute exacerbation of IPF with corticosteroids, oxygen supplementation, mechanical ventilation, pulmonary rehabilitation, and lung transplantation was not a priority in this guideline.
This guideline states that it does not provide recommendations for 1 treatment regimen over another. With the exception of the recommendation against using prednisone with azathioprine and N-acetylcysteine, the guideline does not provide suggestions for or against combination regimens or sequential therapies.
Instead, the guideline continued, strong or conditional ratings must be weighed individually, factoring in all components used to determine the grade of the recommendation, “including the confidence in effect estimates, outcomes studies, desirable and undesirable consequences of treatment, cost of treatment, implications of treatment on health equity, and feasibility of treatment.”