Joint Guidelines Updated for Idiopathic, Progressive Pulmonary Fibrosis

The American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and Asociación Latinoamericana de Tórax have issued an update to their joint guidelines for the diagnosis and management of adult patients with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF), as well as pulmonary fibrosis in patients with other interstitial lung diseases.

The guidelines were issued by a multidisciplinary committee of experts in interstitial lung disease as well as methodologists and patient representatives. They updated radiological and histopathological criteria for IPF and PPF by consensus. In addition, they used systematic reviews and evidence-based recommendations based on the GRADE approach to answer questions about transbronchial lung cryobiopsy, genomic classifier testing, antacids, and antireflux surgery for IPF and about nintedanib and pirfenidone for PPF.

The group conditionally recommended transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. However, they made no recommendation regarding genomic classifier testing. The group also conditionally recommended against antacid medication and antireflux surgery for IPF.

Furthermore, the group defined PPF as meeting at least 2 of the following 3 criteria within the last year: worsening symptoms, radiological progression, and physiological progression. In addition to meeting 2 of 3 of these criteria, patients with an interstitial lung disease other than IPF must have no alternative explanation for the worsening symptoms or progression. The group also conditionally recommended nintedanib for PPF and recommended additional research on pirfenidone for PPF.

“These recommendations are not mandates because they cannot account for all unique clinical circumstances, and they should be revisited as new evidence is published,” authors added.

—Ellen Kurek

Reference:

Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Resp Crit Care Med. 2022;205(9):e18e47. doi:10.1164/rccm.202202-0399ST