J Korean Med Assoc.  2020 Mar;63(3):159-168. 10.5124/jkma.2020.63.3.159.

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

• Kang HR
• Choi SM

• KMID: 2471864
• DOI: http://doi.org/10.5124/jkma.2020.63.3.159

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.

Keyword

Idiopathic pulmonary fibrosis; Idiopathic interstitial pneumonias; Interstitial lung diseases

MeSH Terms

Aged
Cough
Diagnosis*
Dyspnea
Fibrosis
Humans
Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis*
Inflammation
Lung
Lung Diseases, Interstitial*
Prognosis
Wound Healing

Figure

• 

  Figure 1. Classification of interstitial lung diseases (ILDs). IIP, interstitial idiopathic pneumonia.

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  Figure 2. Pathophysiology of idiopathic pulmonary fibrosis (IPF). (A) Normal lung and (B) IPF lung. Drawn by authors.

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  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.

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  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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