Recommendations updated for idiopathic pulmonary fibrosis

In a clinical practice guideline issued by the American Thoracic Society, together with the European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax, updated recommendations are presented for the diagnosis and management of idiopathic pulmonary fibrosis (IPF); the updated guideline was published in the May 1 issue of the American Journal of Respiratory and Critical Care Medicine.

Ganesh Raghu, M.D., from the University of Washington in Seattle, and colleagues updated prior IPF guidelines and addressed the progression to pulmonary fibrosis among patients with other interstitial lung diseases (ILDs).

The researchers made a conditional recommendation with regard to transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy at centers with appropriate expertise for patients with ILD of undetermined type. No recommendation was made in favor of or against genomic classifier testing for diagnosing usual interstitial pneumonia in patients with ILD of undetermined type. For the treatment of IPF, conditional recommendations were made against antacid medication and antireflux surgery. Progressive pulmonary fibrosis was defined as meeting two of three criteria of worsening symptoms, radiologic progression, and physiologic progression within the previous year, with no alternative explanation among patients with an ILD other than IPF. Nintedanib was conditionally recommended, and further research into pirfenidone was recommended.

"Making an accurate diagnosis of IPF and monitoring disease progression in fibrotic ILD other than IPF is essential for consideration of prompt treatment intervention for PPF," Raghu said in a statement.

Several authors disclosed financial ties to pharmaceutical companies, including Boehringer Ingelheim and Genentech, the manufacturers of nintedanib and pirfenidone, respectively.

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