Abstract
In 2022, the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Associatión Latinoamericana de Tórax (ALAT) produced an updated clinical practice guideline on the diagnosis and management of idiopathic pulmonary fibrosis (IPF), also defining and making treatment recommendations for progressive pulmonary fibrosis (PPF). In this review, we summarize and highlight aspects of this guideline that are most relevant to Canadians. New recommendations in IPF include the use of transbronchial lung cryobiopsy as an alternative to surgical lung biopsy and a recommendation against antacid medications in asymptomatic patients. The guideline also recommends treating PPF with nintedanib, an antifibrotic currently approved for IPF. The guideline definition of PPF should be adopted in Canada, although with flexibility in physiologic parameters of the PPF definition and increased support for community respirologists to care for these patients. Although pirfenidone was not recommended in the guideline for the treatment of PPF, there are multiple clinical trials that support its use as a second-line treatment for PPF. It is imperative that Canadian resources continue to support knowledge generation of this complex group of patients and that Canadians continue to have ongoing involvement in the development of future international clinical practice guidelines.
Published Version
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