Description
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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, Comparison 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.
Faculdade de Medicina de Ribeirão Preto Universidade de São Paulo
Escola Paulista de Medicina Universidade Federal de São Paulo
Faculdade de Medicina Universidade de São Paulo
Faculdade de Medicina de Botucatu Universidade Estadual Paulista - UNESP - Botucatu (SP) Brasil
Universidade Católica de Brasília
Hospital Geral Roberto Santos
Universidade Federal do Ceará
Universidade Federal de Minas Gerais
Universidade Federal de Goiás
Universidade de Brasília - UnB - Brasília (DF) Brasil
Hospital do Servidor Público Estadual de São Paulo
Universidade Federal de Ciências da Saúde de Porto Alegre
Universidade Federal de Santa Catarina - UFSC - Florianópolis (SC) Brasil
Faculdade de Medicina de Botucatu Universidade Estadual Paulista - UNESP - Botucatu (SP) Brasil
