Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics

Idiopathic pulmonary fibrosis is a chronic disease of unknown etiology that usually has a progressive course and is commonly associated with a poor prognosis. The main symptoms of idiopathic pulmonary fibrosis, including progressive dyspnea and dry cough, are often nonspecific. Chest high-resolution computed tomography is the primary modality used in the initial assessment of patients with suspected idiopathic pulmonary fibrosis and may have considerable influence on subsequent management decisions. The main role of computed tomography is to distinguish chronic fibrosing lung diseases with a usual interstitial pneumonia pattern from those presenting with a non-usual interstitial pneumonia pattern, suggesting an alternative diagnosis when possible. A usual interstitial pneumonia pattern on chest tomography is characterized by the presence subpleural and basal predominance, reticular abnormality honeycombing with or without traction bronchiectasis, and the absence of features suggestive of an alternative diagnosis. Idiopathic pulmonary fibrosis can be diagnosed according to clinical and radiological criteria in approximately 66.6% of cases. Confirmation of an idiopathic pulmonary fibrosis diagnosis is challenging, requiring the exclusion of pulmonary fibroses with known causes, such as asbestosis, connective tissue diseases, drug exposure, chronic hypersensitivity pneumonitis, and other forms of idiopathic interstitial pneumonitis. The histopathological hallmark of usual interstitial pneumonia is a heterogeneous appearance, characterized by areas of fibrosis with scarring and honeycombing alternating with areas of less affected or normal parenchyma. The aim of this article was to review the clinical, radiological, and pathological features of idiopathic pulmonary fibrosis and of diseases that might mimic idiopathic pulmonary fibrosis presentation

Survival in idiopathic pulmonary fibrosis - cytotoxic agents compared to corticosteroids

Study objective: To compare the survival of patients with IPF treated retrospectively with corticosteroids atone, to survival of patients treated with immunosuppressive and corticosteroids combined. Design: Non-randomized retrospective cohort study.Setting: Three tertiary centers in Brazil.Patients: Eighty-two IPF patients were included. the diagnosis was confirmed by open lung biopsy in 48. Patients received either corticosteroids alone (group 1) or cytotoxic agents in addition to corticosteroids (group 11).Measurements and results: the primary end-point was mortality. Secondary outcome included longitudinal changes in FVC. Mean age was 66+8 years. FVC was 71 +/- 17% of predicted. There were 48 deaths during the study period (59%), 44 secondary to respiratory causes. From preliminary univariate analysis, for the group as a whole, worse survival was found to be associated with FVC% = 70% and = 70% (Log Rank = 6.84, P = 0.009).Conclusions: the combination of immunosuppressive agents and prednisone results in better survival when compared to prednisone alone in patients with IPF the benefit seems to occur only in patients with less severe disease, as reflected by FVC >= 70%. (c) 2005 Elsevier B.V. All rights reserved.Universidade Federal de São Paulo, Dept Pulmonol, Paulista Sch Med, BR-04082001 São Paulo, BrazilUniversidade Federal de São Paulo, Dept Pulmonol, Paulista Sch Med, BR-04082001 São Paulo, BrazilWeb of Scienc

Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.As doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.Universidade de São Paulo Faculdade de Medicina Hospital das ClínicasUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Curso de Pós-Graduação de Doenças Pulmonares IntersticiaisUniversidade Federal de Ciências da Saúde de Porto AlegreSanta Casa de Porto Alegre Ambulatório de Doenças IntersticiaisSES Hospital Regional da Asa Norte Serviço de Doenças TorácicasFundação Jorge Duprat Figueiredo de Segurança e Medicina do Trabalho Serviço de MedicinaUniversidade Estadual de Campinas Faculdade de Ciências Médicas Departamento de Clínica MédicaUniversidade Federal do Estado do Rio de JaneiroUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de PatologiaHospital do Servidor Público Estadual de São Paulo Serviço de Anatomia PatológicaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaUniversidade de São Paulo Faculdade de Medicina de Ribeirão Preto Divisão de PneumologiaUniversidade Federal de Santa Catarina Departamento de Clínica MédicaUniversidade Federal de Santa Catarina Hospital UniversitárioHospital de Messejana Ambulatório de Doenças IntersticiaisHospital do Servidor Público Estadual de São Paulo Ambulatório de Doenças IntersticiaisUniversidade de São Paulo Faculdade de Medicina Instituto do CoraçãoUniversidade Federal FluminenseUniversidade de São Paulo Faculdade de MedicinaHospital Sírio Libanês Núcleo Avançado de TóraxUniversidade Federal da BahiaHospital do Servidor Público Estadual de São PauloHospital do Câncer Antônio Cândido CamargoUNIFESP, EPM, Curso de Pós-Graduação de Doenças Pulmonares IntersticiaisUNIFESP, EPM, Depto. de PatologiaUNIFESP, EPMSciEL

Highlights of the Brazilian Thoracic Association guidelines for interstitial lung diseases

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.As doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.28229

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

A fibrose pulmonar idiopática (FPI) é uma forma de pneumopatia intersticial crônica fibrosante de causa desconhecida, que acomete preferencialmente homens idosos, com história atual ou pregressa de tabagismo. Mesmo sendo uma doença incomum, ela assume grande importância devido a sua gravidade e prognóstico reservado. Nas últimas décadas, diversas modalidades terapêuticas farmacológicas foram investigadas para o tratamento dessa doença, de tal modo que conceitos clássicos vêm sendo revisados. O objetivo destas diretrizes foi definir recomendações brasileiras baseadas em evidências em relação ao emprego de agentes farmacológicos no tratamento da FPI. Procurou-se fornecer orientações a questões de ordem prática, enfrentadas pelos clínicos no seu cotidiano. As perguntas PICO (acrônimo baseado em perguntas referentes aos Pacientes de interesse, Intervenção a ser estudada, Comparação da intervenção e Outcome [desfecho] de interesse) abordaram aspectos relativos ao uso de corticosteroides, N-acetilcisteína, tratamento medicamentoso do refluxo gastroesofágico, inibidores dos receptores da endotelina, inibidores da fosfodiesterase-5, pirfenidona e nintedanibe. Para a formulação das perguntas PICO, um grupo de especialistas brasileiros atuantes na área foi reunido, sendo realizada uma extensa revisão bibliográfica sobre o tema. As revisões sistemáticas com meta-análises previamente publicadas foram analisadas quanto à força das evidências compiladas e, a partir daí, foram concebidas recomendações seguindo a metodologia Grading of Recommendations Assessment, Development and Evaluation. Os autores acreditam que o presente documento represente um importante avanço a ser incorporado na abordagem de pacientes com FPI, objetivando principalmente favorecer seu manejo, e pode se tornar uma ferramenta auxiliar na definição de políticas públicas relacionadas à FPI.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