Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Abstract\r\nBackground: This American Thoracic Society, European\r\nRespiratory Society, Japanese Respiratory Society, and\r\nAsociacion Latinoamericana de T orax guideline updates \r\nprior idiopathic pulmonary fibrosis (IPF) guidelines\r\nand addresses the progression of pulmonary fibrosis in\r\npatients with interstitial lung diseases (ILDs) other\r\nthan IPF.\r\nMethods: A committee was composed of multidisciplinary\r\nexperts in ILD, methodologists, and patient representatives.\r\n1) Update of IPF: Radiological and histopathological criteria\r\nfor IPF were updated by consensus. Questions about\r\ntransbronchial lung cryobiopsy, genomic classifier testing,\r\nantacid medication, and antireflux surgery were informed by\r\nsystematic reviews and answered with evidence-based\r\nrecommendations using the Grading of Recommendations,\r\nAssessment, Development and Evaluation (GRADE) approach.\r\n2) Progressive pulmonary fibrosis (PPF): PPF was defined, and\r\nthen radiological and physiological criteria for PPF were\r\ndetermined by consensus. Questions about pirfenidone and\r\nnintedanib were informed by systematic reviews and answered\r\nwith evidence-based recommendations using the GRADE\r\napproach.\r\nResults: 1) Update of IPF: A conditional recommendation was\r\nmade to regard transbronchial lung cryobiopsy as an acceptable\r\nalternative to surgical lung biopsy in centers with appropriate\r\nexpertise. No recommendation was made for or against genomic\r\nclassifier testing. Conditional recommendations were made\r\nagainst antacid medication and antireflux surgery for the\r\ntreatment of IPF. 2) PPF: PPF was defined as at least two of\r\nthree criteria (worsening symptoms, radiological progression,\r\nand physiological progression) occurring within the past year\r\nwith no alternative explanation in a patient with an ILD other\r\nthan IPF. A conditional recommendation was made for\r\nnintedanib, and additional research into pirfenidone was\r\nrecommended.\r\nConclusions: The conditional recommendations in this\r\nguideline are intended to provide the basis for rational, informed\r\ndecisions by clinicians.\r\nKeywords: idiopathic pulmonary fibrosis; progressive pulmonary\r\nfibrosis; radiology; histopathology