Pharmacological treatment of pulmonary fibrosis

Won-Il Choi

doi:10.5124/jkma.2020.63.1.47

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Choi: Pharmacological treatment of pulmonary fibrosis

Pharmacotherapeutics

J Korean Med Assoc 2020;63(1):47-55.

Published online: 17 January 2020

DOI: https://doi.org/10.5124/jkma.2020.63.1.47

Pharmacological treatment of pulmonary fibrosis

Won-Il Choi, MD

Department of Internal Medicine, Myongji Hospital, Hanyang University College of Medicine, Goyang, Korea

Corresponding author: Won-Il Choi E-mail: choi_wi@hanmail.net

Revised 31 October 2019 Accepted 25 November 2019

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

Idiopathic pulmonary fibrosis (IPF) is a condition that has been described as alveolar collapse and thickening, which correlate with dysregulated surfactant production and injury to type 2 alveolar cells. As resolution of chest computed tomography has improved, especially with the development of high-resolution computed tomography (HRCT), the diagnostic measures adopted for pulmonary fibrosis has gradually shifted from biopsy to HRCT. This shift towards HRCT has aided in diagnostic evaluation and detection of the therapeutic and adverse effects of drugs for pulmonary fibrosis. Further, after the endpoint was changed to forced vital capacity, significant improvements are being observed in clinical trial outcomes. Currently active clinical trials are replacing lung biopsy with HRCT. In 2014, pirfenidone and nintedanib gained approval for tandem use in patients with IPF. These drugs were found to not only reduce the progression of pulmonary fibrosis, but also the acute exacerbation and mortality associated with the condition. These drugs showed consistent benefits regardless of the severity of patients’ symptoms. Additionally, both nintedanib and pirfenidone were found to be effective in patients with advanced pulmonary fibrosis that was not classified as IPF. Nintedanib has been shown to reduce forced vital capacity in interstitial lung diseases associated with systemic sclerosis. In the next three to five years, many changes in treatment are expected, not only for IPF, but also for the entire spectrum of pulmonary fibrotic diseases. Pirfenidone and nintedanib are now considered standard treatments for IPF and few other fibrotic lung diseases. Clinicians treating patients with pulmonary fibrosis should keep themselves updated with the results of clinical trials that are currently underway.

Keywords: Idiopathic pulmonary fibrosis, Interstitial lung diseases, Nintedanib, Pirfenidone

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Figure 1.

Pirfenidone prescription algorithm based on the National Insurance Service payment in 2019. UIP, usual interstitial pneumonia; HRCT, high-resolution computed tomography; FVC, forced vital capacity; DLco, carbon monoxide diffusing capacity.

Table 1.

Therapeutic effects of major drugs on IPF and other fibrotic interstitial lung diseases

Fibrotic lung diseases	Drugs
Fibrotic lung diseases	Pirfenidone	Nintedanib	Sildenafil
IPF
Mild, FVC predicted ≥90%	+	+
Moderate, FVC predicted ≥50% <90%	++	++
Severe, FVC predicted <50%	+	+
Possible usual interstitial pneumonia in HRCT^a)	?	+
Unclassifiable ILD	+	+
IPF with right ventricle dysfunction	?	+	+^b)
Chronic hypersensitivity pneumonitis	?	+
Nonspecific interstitial pneumonia	?	+
Systemic sclerosis-associated ILD	?	++

IPF, idiopathic pulmonary fibrosis; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease. +, proven effectiveness in studies other than large randomized trials; ++, primary end point met in a large randomized trial; ?, not yet studied.

^a) Possible usual interstitial pneumonia is now renamed to probable usual interstitial pneumonia.

^b) Requires co-administration with nintedanib.

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