Progressive Pulmonary Fibrosis: Should the Timelines Be Taken Out of the Definition?

• Published in: American journal of respiratory and critical care medicine Vol. 206; no. 10; pp. 1293 - 1294
• Main Authors: Cottin, Vincent, Brown, Kevin K, Flaherty, Kevin R, Wells, Athol U
• Format: Journal Article
• Language: English
• Published: United States American Thoracic Society 15.11.2022
• Subjects: Chronic obstructive pulmonary disease; Cystic fibrosis; Humans; Idiopathic Pulmonary Fibrosis - diagnosis; Lung diseases; Pulmonary fibrosis; Pulmonary hypertension
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.202206-1143LE

An important section of the new ATS/ERS/JRS/ALAT (American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Asociacion Latinoamericana del Torax) guideline on pulmonary fibrosis proposes a definition and criteria for the diagnosis of progressive pulmonary fibrosis (PPF), a different type of pulmonary fibrosis than idiopathic pulmonary fibrosis. Clinical studies evaluating the efficacy and acceptability of antifibrotic treatment in PPF despite management have utilized a variety of disease progression definitions. (also known as interstitial lung disease with a progressive fibrotic phenotype). The authors of the guidelines suggest the term PPF (which was initially put forth in a review paper and setting standards that, if widely embraced, will aid in establishing a uniform standard for research in the subject.