Tuberc Respir Dis.  2019 Apr;82(2):102-117. 10.4046/trd.2018.0091.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 2. Idiopathic Pulmonary Fibrosis

• Lee SH
• Yeo Y
• Kim TH
• Lee HL
• Lee JH
• Park YB
• Park JS
• Kim YH
• Song JW
• Jhun BW
• Kim HJ
• Park J
• Uh ST
• Kim YW
• Kim DS
• Park MS
• Korean Interstitial Lung Diseases Study Group

• KMID: 2453980
• DOI: http://doi.org/10.4046/trd.2018.0091

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia, which presents with a progressive worsening dyspnea, and thus a poor outcome. The members of the Korean Academy of Tuberculosis and Respiratory Diseases as well as the participating members of the Korea Interstitial Lung Disease Study Group drafted this clinical practice guideline for IPF management. This guideline includes a wide range of topics, including the epidemiology, pathogenesis, risk factors, clinical features, diagnosis, treatment, prognosis, and acute exacerbation of IPF in Korea. Additionally, we suggested the PICO for the use of pirfenidone and nintendanib and for lung transplantation for the treatment of patients with IPF through a systemic literature review using experts' help in conducting a meta-analysis. We recommend this guideline to physicians, other health care professionals, and government personnel in Korea, to facilitate the treatment of patients with IPF.

Keyword

Idiopathic Pulmonary Fibrosis; Diagnosis; Disease Management; Korea

MeSH Terms

Delivery of Health Care
Diagnosis*
Disease Management
Dyspnea
Epidemiology
Humans
Idiopathic Pulmonary Fibrosis*
Korea
Lung Diseases, Interstitial*
Lung Transplantation
Prognosis
Risk Factors
Tuberculosis

Figure

• 

  Figure 1 High resolution computed tomography images demonstrating usual interstitial pneumonia (arrows) (A) and possible usual interstitial pneumonia (arrows) (B) (Courtesy from Ulsan College of Medicine, Asan Medical Center, Internal Medicine, Prof. Song JW).

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  Figure 2 Surgical lung biopsy specimen demonstrating usual interstitial pneumonia pattern (Courtesy from Yonsei University, College of Pathology Prof. Sim HS).

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  Figure 3 Forest plot of the risk difference of percentage of study population with forced vital capacity decreased by more than 10%. CI: confidence interval.

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  Figure 4 Difference in annual forced vital capacity decline rate between nintedanib and placebo groups. CI: confidence interval.

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  Figure 5 Difference in survival between lung transplantation and placebo groups. OR: odds ratio; CI: confidence interval.

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  Figure 6 Diagnostic algorism of acute respiratory deterioration in idiopathic pulmonary fibrosis patients. IPF: idiopathic pulmonary fibrosis; GGO: ground glass opacity; CT: computed tomography.

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