Idiopathic pulmonary fibrosis: pathogenesis and management

Respiratory Research
Giacomo SgallaLuca Richeldi

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. This review will present the substantial advances achieved in the understanding of IPF pathogenesis and in the therapeutic options that can be offered to patients, and will address the issues regarding diagnosis and management that are still open. Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF. Sustained alveolar epithelial micro-injury and activation has been recognised as the trigger of several biological events of disordered repair occurring in genetically susceptible ageing individuals. Despite multidisciplinary team discussion has demonstrated to increase diagnostic accuracy, patients can still remain unclassified when the current diagnostic criteria are strictly applied, requiring the identification of a Usual Interstitial Pattern either on high-resolution computed tomography scan or lung biopsy. Outstanding achievements have been made in the management of these patients, as nintedanib and pirfenidone consist...Continue Reading

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Methods Mentioned

BETA
bronchoalveolar lavage
bronchoalveolar
lavage
biopsy
lung
imaging technique
cognitive behavioural therapy
pharmacotherapies

Clinical Trials Mentioned

NCT02606877

Software Mentioned

PROFILE
IPF
INPULSIS

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