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Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Department of Respiratory Medicine, Okinawa Chubu Hospital, 904-2293 Miyazato 281, Uruma City, Okinawa, Japan
Medicina 2019, 55(3), 70;
Received: 17 December 2018 / Revised: 18 February 2019 / Accepted: 14 March 2019 / Published: 16 March 2019
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis Research)
PDF [263 KB, uploaded 16 March 2019]


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. View Full-Text
Keywords: acute exacerbation; consolidation; GGO; HRCT; idiopathic; IPF; LDH; nintedanib; pirfenidone; triggered acute exacerbation; consolidation; GGO; HRCT; idiopathic; IPF; LDH; nintedanib; pirfenidone; triggered
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Kishaba, T. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Medicina 2019, 55, 70.

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