2022 update on clinical practice guidelines for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

The 2022 update on guidelines for idiopathic pulmonary fibrosis (IPF) by ATS/ERS/JRS/ALAT 1 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 Respir Crit Care Med. 2022; 205: e18-47 Crossref PubMed Scopus (82) Google Scholar consists of two parts. The first part is an update on the diagnosis and treatment of IPF and the second deals with the important issue of progressive pulmonary fibrosis (panel). PanelHighlights of the 2022 ATS/ERS/JRS/ALAT Clinical Practice Guideline on IPF and PPFDiagnosis of IPF •The presence of the probable UIP pattern can support the diagnosis of IPF in the appropriate clinical setting within the context of multidisciplinary discussion, obviating the need for tissue confirmation •Transbronchial cryobiopsy is an acceptable alternative to surgical lung biopsy in patients with ILD of undetermined type, in medical centres with appropriate expertise •No recommendation is made regarding the addition of genomic classifier testing for the purpose of diagnosing UIP in patients with undetermined ILD undergoing transbronchial forceps biopsy Treatment of IPF •Patients with IPF should not be treated with antacid medication, with the aim of improving respiratory outcomes •Patients with IPF should not be referred for anti-reflux surgery, with the aim of improving respiratory outcomes Progressive pulmonary fibrosis •The concept of PPF can be applied to patients with fibrotic ILD of known or unknown cause other than IPF •PPF is defined on the basis of the presence of at least two of the following three criteria occurring within one year: •Worsening respiratory symptoms •Physiological evidence of disease progression, including one or both of: •Absolute decline in forced vital capacity of at least 5% predicted •Absolute decline in diffusing capacity for carbon monoxide of at least 10% predicted •Radiological evidence of disease progressionNintedanib is suggested for the treatment of PPF in patients unresponsive to standard management, and further research of both nintedanib and pirfenidone is recommended IPF=idiopathic pulmonary fibrosis. ILD=interstitial lung disease. PPF=progressive pulmonary fibrosis. Diagnosis of IPF •The presence of the probable UIP pattern can support the diagnosis of IPF in the appropriate clinical setting within the context of multidisciplinary discussion, obviating the need for tissue confirmation •Transbronchial cryobiopsy is an acceptable alternative to surgical lung biopsy in patients with ILD of undetermined type, in medical centres with appropriate expertise •No recommendation is made regarding the addition of genomic classifier testing for the purpose of diagnosing UIP in patients with undetermined ILD undergoing transbronchial forceps biopsy Treatment of IPF •Patients with IPF should not be treated with antacid medication, with the aim of improving respiratory outcomes •Patients with IPF should not be referred for anti-reflux surgery, with the aim of improving respiratory outcomes Progressive pulmonary fibrosis •The concept of PPF can be applied to patients with fibrotic ILD of known or unknown cause other than IPF •PPF is defined on the basis of the presence of at least two of the following three criteria occurring within one year: •Worsening respiratory symptoms •Physiological evidence of disease progression, including one or both of: •Absolute decline in forced vital capacity of at least 5% predicted •Absolute decline in diffusing capacity for carbon monoxide of at least 10% predicted •Radiological evidence of disease progressionNintedanib is suggested for the treatment of PPF in patients unresponsive to standard management, and further research of both nintedanib and pirfenidone is recommended IPF=idiopathic pulmonary fibrosis. ILD=interstitial lung disease. PPF=progressive pulmonary fibrosis.