New Insights on Mechanisms and Treatment in Idiopathic Pulmonary Fibrosis
DOI:
https://doi.org/10.12974/2312-5470.2016.03.2Keywords:
Idiopathic pulmonary fibrosis, usual interstitial pneumonia, nintedanib, pirfenidone.Abstract
Interstitial lung disease (ILD) represents a large group of diseases characterized by various degrees of inflammation and fibrosis, with progressive scarring of the interstitium, the tissue around the air sacs, leading to lung stiffness and restrictive pattern. Depending on the main pathological process, ILD extends from inflammatory type diseases, follicular bronchiolitis being the most representative, to fibrotic type diseases, with idiopathic pulmonary fibrosis (IPF) being the most common and studied. The aim of the article is to review the current knowledge on pathogenesis, diagnosis and treatment in IPF based on research results and recent trials data. Nintedanib and pirfenidone are pointed out as the only two conditional recommended drugs for IPF by the current international guidelines.
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