José Baddini-Martinez1,2, Juliana Ferreira3, Suzana Tanni4, Luis Renato Alves1, Benedito Francisco Cabral Junior5, Carlos Roberto Ribeiro Carvalho3, Talita Jacon Cezare4, Claudia Henrique da Costa6, Marcelo Basso Gazzana7, Sérgio Jezler8, Ronaldo Adib Kairalla3, Leticia Kawano-Dourado9, Mariana Silva Lima10, Eliane Mancuzo11, Maria Auxiliadora Carmo Moreira12, Marcelo Palmeira Rodrigues13, Silvia Carla Sousa Rodrigues14, Adalberto Sperb Rubin15, Rogério Lopes Rufino6, Leila John Marques Steidle16, Karin Storrer17, Bruno Guedes Baldi3
J Bras Pneumol.2020;46(2):e20190423
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.
Keywords: Idiopathic pulmonary fibrosis; GRADE approach; Pulmonary fibrosis/drug therapy; Practice guideline.