ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.
Subject(s)
Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial , Humans , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/drug therapy , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/drug therapyABSTRACT
ABSTRACT Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.
RESUMO A fibrose pulmonar idiopática (FPI) é uma doença pulmonar crônica devastadora sem uma causa claramente reconhecida, que está na vanguarda de novos algoritmos de diagnóstico e do desenvolvimento de tratamentos que levaram a uma mudança no cuidado desses pacientes na última década, influenciando de fato o manejo de doenças pulmonares intersticiais fibróticas além da própria FPI. A apresentação clínica, a fisiopatologia e os critérios diagnósticos são brevemente abordados neste artigo de revisão. Além disso, as evidências sobre o uso de antifibróticos além dos cenários de ensaios clínicos, o impacto de comorbidades e abordagens terapêuticas, além dos tratamentos farmacológicos são discutidos detalhadamente.
