An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline
The update that changed what idiopathic pulmonary fibrosis is treated with. Until 2011 the disease was managed as an inflammatory one, with prednisone, azathioprine and N-acetylcysteine given together; PANTHER-IPF stopped that arm early for death and hospitalisation, and this document recommends against the combination. Recommendations against warfarin, ambrisentan and imatinib follow the same logic: each had a plausible mechanism and a trial that found harm or nothing. What is left is antifibrotic: conditional recommendations for nintedanib and for pirfenidone, both of which slow the fall in forced vital capacity. Neither was shown to extend life in the trials the panel had, and the guideline is explicit that a conditional recommendation is what the evidence supports. The register carries the three trials that decided each of those questions, and INBUILD, which came later and extended nintedanib beyond IPF to progressive fibrosis of other causes.
This guideline cites 3 of the 35 landmark trials in our Pulmonology register.
Antifibrotic therapy2
Against immunosuppression1
- 2012PANTHER-IPFPulmonology · ILD · Immunosuppression — IPFPrednisone, Azathioprine and N-Acetylcysteine in IPF