Diagnosis and management of idiopathic pulmonary fibrosis
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Authors
Joshi, v
Nagi, Z
Issue Date
2022
Type
Scientific Paper
Language
Keywords
Research Subject Categories::MEDICINE::Dermatology and venerology,clinical genetics, internal medicine::Internal medicine::Lung diseases
Alternative Title
Abstract
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Its signs and symptoms are relatively non-specific, and patients often present with chronic cough, progressive dyspnea, resting or exertional hypoxemia, and inspiratory crackles on lung auscultation. Definitive diagnosis requires the exclusion of known causes of pulmonary fibrosis and identification of the usual interstitial pneumonia (UIP) pattern of disease either on high-resolution computed tomography (HRCT) scan of the chest or on surgical lung biopsy. Multidisciplinary discussion involving pulmonologists, radiologists, and pathologists with expertise in the diagnosis of IPF and other forms of interstitial lung disease is recommended and often required. Management focuses on anti-fibrotic therapy and early referral to lung transplant centers for those who are candidates. This review will discuss the current recommendations for the diagnosis, prognostication, and management of patients with IPF.
Keywords: diffuse parenchymal lung diseases (DPLD); cryptogenic fibrosing alveolitis; idiopathic interstitial pneumonia (IIP); idiopathic pulmonary fibrosis (IPF); interstitial lung disease (ILD); usual interstitial pneumonia (UIP).
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Citation
Munchel JK, Shea BS. Diagnosis and Management of Idiopathic Pulmonary Fibrosis. R I Med J (2013). 2021 Sep 1;104(7):26-29. PMID: 34437662.