ATS issues new clinical practice guideline on IPF, progressive fibrotic ILDs

SAN FRANCISCO — A session at the American Thoracic Society International Conference highlighted a new clinical practice guideline addressing idiopathic pulmonary fibrosis and progressive pulmonary fibrosis in adults.

The new guidance comes nearly 4 years after the last guidance on the diagnosis and management of IPF, according to the ATS.

In addition to providing guidance to clinicians who care for adults with IPF, the new guideline addresses progressive pulmonary fibrosis in ILDs other than IPF, provides a new definition to recognize progressive pulmonary fibrosis, and offers recommendations for treatment of ILDs other than IPF that manifest as progressive pulmonary fibrosis, according to a press release.

Currently, most clinicians diagnose IPF using clinical, bronchoalveolar cellular and radiological features. This trend prompted a reevaluation of current clinical practice guidelines to refine usual interstitial pneumonia (UIP) patterns and provide current evidence-based guidance to ascertain UIP diagnosis in smaller transbronchial lung biopsies obtained without surgical lung biopsy.

The ATS, in collaboration with the European Respiratory Society, Japanese Respiratory Society and the Asociación Latinoamericana de Tórax, convened a committee of multidisciplinary ILD experts, methodologists and patient representatives. The committee developed the following evidence-based recommendations for IPF:

• Transbronchial lung cryobiopsy should be considered an acceptable alternative to surgical lung biopsy for making a histopathological diagnosis in patients with ILD of undetermined type in centers with expertise.
• There is no recommendation either for or against genomic classified testing to diagnose usual interstitial pneumonia (UIP) in patients with ILD of undetermined type undergoing transbronchial forceps biopsy. There was insufficient agreement among the committee members for this recommendation.

Increasingly, the trend is to diagnose IPF using clinical, bronchoalveolar cellular and radiological features, and in patients who need histopathology diagnosis, using smaller lung biopsies with less-invasive procedures other than surgical lung biopsy. This trend prompted the committee to refine the patterns of UIP and provide the current evidence-based guidance for ascertaining the diagnosis of UIP in smaller transbronchial lung biopsies obtained without surgical lung biopsy, according to the release.

The following recommendations were made for treating abnormal GERD to improve respiratory outcomes:

• Antacid medication for the purpose of improving respiratory outcomes is not recommended for patients with IPF.
• Patients with IPF should not be referred for anti-reflux surgery for the purpose of improving respiratory outcomes.

The following recommendations were made for treating non-IPF progressive pulmonary fibrosis:

• Further research is needed on the efficacy, effectiveness and safety of pirfenidone in patients with non-IPF progressive pulmonary fibrosis and also in patients with specific types of ILD that manifest progressive pulmonary fibrosis.
• Nintedanib (Ofev, Boehringer Ingelheim) is recommended for the treatment of progressive pulmonary fibrosis in patients who failed standard management.

“While this was in the works, a clinical trial reporting a beneficial effect of antifibrotic medication in interstitial lung diseases other than IPF that manifest progressive pulmonary fibrosis prompted a paradigm shift toward an en bloc approach to antifibrotic therapy,” Ganesh Raghu, MD, chair of the clinical practice guidelines committee, professor of medicine in laboratory medicine and pathology at the University of Washington, and director of the Center for Interstitial Lung Disease at the University of Washington Medical Center, Seattle, said during a presentation at the ATS International Conference. “Given the importance and timeliness of the issue, the guideline committee was approved to expand its scope to also define progression of pulmonary fibrosis and to decide whether the en bloc approach to antifibrotic therapy should continue or whether therapy should be restricted to specific types of progressive ILD.”

The guideline was published in the American Journal of Respiratory and Critical Care Medicine.

References:

• Raghu G, et al. Am J Respir Crit Care Med. 2022;doi:10.1164/rccm.202202-0399ST.
• New clinical practice guidelines on idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. Published May 4, 2022. Accessed May 13, 2022.