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

2020; Sociedade Brasileira de Pneumologia e Tisiologia; Volume: 46; Issue: 2 Linguagem: Inglês

10.36416/1806-3756/e20190423

1806-3756

José Antônio Baddini Martínez, Juliana Carvalho Ferreira, Suzana Érico Tanni, Luis Renato Alves, Benedito Francisco Cabral, Carlos Roberto Ribeiro de Carvalho, Talita Jacon Cezare, Cláudia Henrique da Costa, Marcelo Basso Gazzana, Sérgio Jezler, Ronaldo Adib Kairalla, Letícia Kawano-Dourado, Mariana Silva Lima, Eliane Viana Mancuzo, Maria Auxiliadora Carmo Moreira, Marcelo Palmeira Rodrigues, Sílvia Rodrigues, Adalberto Sperb Rubin, Rogério Rufino, Leila John Marques Steidle, Karin Mueller Storrer, Bruno Guedes Baldi,

Inhalation and Respiratory Drug Delivery

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.