Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Mar 16;55(3):70.
doi: 10.3390/medicina55030070.

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Tomoo Kishaba  1
Affiliations
Review

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Tomoo Kishaba. Medicina (Kaunas). .

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia. Idiopathic pulmonary fibrosis is often seen in elderly men who smoke. A diagnosis of IPF is based on a combination of a detailed clinical history, specific physical examination, laboratory findings, pulmonary function tests, high-resolution computed tomography (HRCT) of the chest, and histopathology. Idiopathic pulmonary fibrosis has a heterogeneous clinical course, from an asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). Acute exacerbation of IPF has several important differential diagnoses, such as heart failure and volume overload. The International Working Group project proposed new criteria for defining AE of IPF in 2016, which divides it into triggered and idiopathic AE. On the basis of these criteria, physicians can detect AE of IPF more easily. The recent international IPF guidelines emphasized the utility of chest HRCT. In addition, two antifibrotic agents have become available. We should focus on both the management and prevention of AE. The diagnostic process, laboratory findings, typical chest imaging, management, and prognosis of AE are comprehensively reviewed in this article.

Keywords: GGO; HRCT; IPF; LDH; acute exacerbation; consolidation; idiopathic; nintedanib; pirfenidone; triggered.

PubMed Disclaimer

Conflict of interest statement

The author declare no conflict of interest.

References

    1. Raghu G., Remy-Jardin M., Myers J.L., Richeldi L., Ryerson C.J., Lederer D.J., Behr J., Cottin V., Danoff S.K., Morell F., et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am. J. Respir. Crit. Care Med. 2018;198:e44–e68. doi: 10.1164/rccm.201807-1255ST. - DOI - PubMed
    1. American Thoracic Society European Respiratory Society Idiopathic pulmonary fibrosis: Diagnosis and treatment. International consensus statement. Pt 1Am. J. Respir. Crit. Care Med. 2000;161:646–664. - PubMed
    1. Murata K., Koga Y., Kasahara N., Hachisu Y., Nunomura S., Nakajima N., Yokoo H., Kaira K., Maeno T., Dobashi K., et al. Accumulation of periostin in acute exacerbation of familial idiopathic pulmonary fibrosis. J. Thorac. Dis. 2018;10:E587–E591. doi: 10.21037/jtd.2018.06.96. - DOI - PMC - PubMed
    1. Okamoto T., Mathai S.K., Hennessy C.E., Hancock L.A., Walts A.D., Stefanski A.L., Brown K.K., Lynch D.A., Cosgrove G.P., Groshong S.D., et al. The relationship between complement C3 expression and the MUC5B genotype in pulmonary fibrosis. Am. J. Physiol. Lung Cell Mol. Physiol. 2018;315:L1–L10. doi: 10.1152/ajplung.00395.2017. - DOI - PMC - PubMed
    1. Dressen A., Abbas A.R., Cabanski C., Reeder J., Ramalingam T.R., Neighbors M., Bhangale T.R., Brauer M.J., Hunkapiller J., Reeder J., et al. Analysis of protein-altering variants in telomerase genes and their association with MUC5B common variant status in patients with idiopathic pulmonary fibrosis: A candidate gene sequencing study. Lancet Respir. Med. 2018;6:603–614. doi: 10.1016/S2213-2600(18)30135-8. - DOI - PMC - PubMed

MeSH terms