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Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

H Alsomali, Evelyn Palmer, Avinash Aujayeb, Wendy Funston

2023Pulmonary Therapy56 citationsDOIOpen Access PDF

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3-5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

Topics & Concepts

NintedanibMedicinePirfenidoneIdiopathic pulmonary fibrosisIntensive care medicineInterstitial lung diseaseEtiologyLung cancerPulmonary function testingDiseaseLife expectancyHigh-resolution computed tomographyLungInternal medicinePopulationEnvironmental healthInterstitial Lung Diseases and Idiopathic Pulmonary FibrosisSarcoidosis and Beryllium Toxicity ResearchRespiratory and Cough-Related Research
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