Brazilian guidelines for the pharmacological treatment of idiopathic pulmonary fibrosis. Official document of the Brazilian Thoracic Association based on the GRADE methodology

Abstract

Idiopathic pulmonary fibrosis (IPF) is a form of chronic interstitial lung disease of unknown cause, which predominantly affects elderly men who are current or former smokers. Even though it is an uncommon disease, it is of great importance because of its severity and poor prognosis. In recent decades, several pharmacological treatment modalities have been investigated for the treatment of this disease, and the classic concepts have therefore been revised. The purpose of these guidelines was to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of IPF in Brazil. We sought to provide guidance on the practical issues faced by clinicians in their daily lives. Patients of interest, Intervention to be studied, C omparison of intervention and Outcome of interest (PICO)-style questions were formulated to address aspects related to the use of corticosteroids, N-acetylcysteine, gastroesophageal reflux medications, endothelin-receptor antagonists, phosphodiesterase-5 inhibitors, pirfenidone, and nintedanib. To formulate the PICO questions, a group of Brazilian specialists working in the area was assembled and an extensive review of the literature on the subject was carried out. Previously published systematic reviews with meta-analyses were analyzed for the strength of the compiled evidence, and, on that basis, recommendations were developed by employing the Grading of Recommendations Assessment, Development and Evaluation approach. The authors believe that the present document represents an important advance to be incorporated in the approach to patients with IPF, aiming mainly to improve its management, and can become an auxiliary tool for defining public policies related to IPF.

Critical appraisal of the quality of clinical practice guidelines for idiopathic pulmonary fibrosis

“…All 12 included CPGs (11 CPGs published in journals and 1 on the NICE website) were published between 2013 and 2020 (2,(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33). All were developed by local or international medical societies.…”

“…All were developed by local or international medical societies. Two CPGs (2,23) were globally developed, and the others came from nine different countries: France, Germany, Spain, Switzerland, Japan, Korea, England, Brazil, and Poland (24)(25)(26)(27)(28)(29)(30)(31)(32)(33). Six CPGs (24,26,28,30,31,33) covered the diagnosis and treatment of IPF, five referred to treatment (23,25,27,29,32), and one referred to diagnosis (2).…”

“…Two CPGs (2,23) were globally developed, and the others came from nine different countries: France, Germany, Spain, Switzerland, Japan, Korea, England, Brazil, and Poland (24)(25)(26)(27)(28)(29)(30)(31)(32)(33). Six CPGs (24,26,28,30,31,33) covered the diagnosis and treatment of IPF, five referred to treatment (23,25,27,29,32), and one referred to diagnosis (2). Six CPGs were updated and developed from original guidelines (2,(23)(24)(25)27,31), three were adapted from other guidelines (26,28,30), and the remaining three were original versions (2,32,33).…”

“…Six CPGs (24,26,28,30,31,33) covered the diagnosis and treatment of IPF, five referred to treatment (23,25,27,29,32), and one referred to diagnosis (2). Six CPGs were updated and developed from original guidelines (2,(23)(24)(25)27,31), three were adapted from other guidelines (26,28,30), and the remaining three were original versions (2,32,33). The ranking systems of evidence quality also varied among these CPGs.…”

“…The ranking systems of evidence quality also varied among these CPGs. The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system ( 34) was adopted to grade evidence quality in eight CPGs (2,23,26,27,29,(31)(32)(33). The Oxford Centre for Evidencebased Medicine Levels of Evidence (35) were adopted for one of the selected guidelines (25), and the remaining three CPGs did not report the criteria they used to grade evidence (24,28,30).…”