Diagnosis and treatment of interstitial lung disease: focusing on idiopathic pulmonary fibrosis

Hye-Rin Kang; Sun Mi Choi

doi:10.5124/jkma.2020.63.3.159

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Kang and Choi: Diagnosis and treatment of interstitial lung disease: focusing on idiopathic pulmonary fibrosis

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J Korean Med Assoc 2020;63(3):159-168.

Published online: 17 March 2020

DOI: https://doi.org/10.5124/jkma.2020.63.3.159

Diagnosis and treatment of interstitial lung disease: focusing on idiopathic pulmonary fibrosis

Hye-Rin Kang, MD, Sun Mi Choi, MD

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea

Corresponding author: Sun Mi Choi E-mail: sunmich81@gmail.com

Revised 7 February 2020 Accepted 3 March 2020

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Interstitial lung disease (ILD) is a rare condition characterized by extensive inflammation and fibrosis mainly involving the pulmonary interstitium or alveoli. Usually, patients with ILD clinically present with chronic cough and exertional dyspnea. ILD is classified into subtypes based on clinical characteristics, detailed history obtained from patients, and radiological, and/or histopathological features. The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD and is associated with poor prognosis. An exclusive diagnosis of IPF requires no known condition causing ILD and typical radiological and/or histopathological features of lung fibrosis. Fibrosis observed in this condition is attributable to repetitive epithelial injury with consequent abnormal wound healing in genetically susceptible and elderly individuals. Currently, pirfenidone and nintedanib are useful disease-modifying agents available to treat IPF. In this article, we review the concept, diagnosis, clinical course, and treatment of ILD.

Keywords: Interstitial lung diseases, Idiopathic interstitial pneumonias, Idiopathic pulmonary fibrosis

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Figure 1.

Classification of interstitial lung diseases (ILDs). IIP, interstitial idiopathic pneumonia.

Figure 2.

Pathophysiology of idiopathic pulmonary fibrosis (IPF). (A) Normal lung and (B) IPF lung. Drawn by authors.

Figure 3.

Typical usual interstitial pneumonia pattern of chest X-ray and high-resolution computed tomography of patient with idiopathic pulmonary fibrosis. Increased extent of diffuse subpleural reticular opacities, traction bronchiectasis and honeycombing in both lungs showing a basilar predominancy, consistent with usual interstitial pneumonia pattern. (A) Chest posteroanterior and (B) high-resolution computed tomography. The patient provided written informed consent for the publication.

Figure 4.

Diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). HRCT, high-resolution computed tomography; IIP, idiopathic interstitial pneumonia; CTD, connective tissue disease; ILD, interstitial lung disease; UIP, usual interstitial pneumonia; BAL, bronchoalveolar lavage; MDD, multidisciplinary discussion.

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