Acute Exacerbations in Patients With Idiopathic Pulmonary Fibrosis

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Abstract

In spite of many studies, the real nature, etiology, pathobiology, and therapy of acute exacerbation (AEx) of idiopathic pulmonary fibrosis (IPF) are not clear. It seemed that AEx-IPF may be an acute acceleration of the underlying fibroproliferative process triggered by various extrinsic or unknown insults in the patients with IPF, who have a predisposition to abnormal wound healing and exaggerated fibrosis. This chapter summarizes the previous studies on etiology/triggering factors, risk factors, prognosis, and therapeutic trials with the introduction of the new consensus definition and diagnostic criteria, which remove “idiopathic” from the 2007 consensus definition, to improve the feasibility of future researches.

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Most cited references 85

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Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis.

Luca Richeldi, Ulrich Costabel, Moises Selman … (2011)

Idiopathic pulmonary fibrosis is a progressive lung disease with a high mortality rate. Because the signaling pathways activated by several tyrosine kinase receptors have been shown to be involved in lung fibrosis, it has been suggested that the inhibition of these receptors may slow the progression of idiopathic pulmonary fibrosis. In a 12-month, phase 2 trial, we assessed the efficacy and safety of four different oral doses of the tyrosine kinase inhibitor BIBF 1120 as compared with placebo in patients with idiopathic pulmonary fibrosis. The primary end point was the annual rate of decline in forced vital capacity (FVC). Secondary end points included acute exacerbations, quality of life (measured with the St. George's Respiratory Questionnaire [SGRQ]), and total lung capacity. A total of 432 patients underwent randomization to receive one of four doses of BIBF 1120 (50 mg once a day, 50 mg twice a day, 100 mg twice a day, or 150 mg twice a day) or placebo. In the group receiving 150 mg of BIBF 1120 twice a day, FVC declined by 0.06 liters per year, as compared with 0.19 liters per year in the placebo group, a 68.4% reduction in the rate of loss with BIBF 1120 (P = 0.06 with the closed testing procedure for multiplicity correction; P = 0.01 with the hierarchical testing procedure). This dose also resulted in a lower incidence of acute exacerbations, as compared with placebo (2.4 vs. 15.7 per 100 patient-years, P = 0.02) and a small decrease in the SGRQ score (assessed on a scale of 0 to 100, with lower scores indicating better quality of life) as compared with an increase with placebo (-0.66 vs. 5.46, P = 0.007). Gastrointestinal symptoms (which led to more discontinuations in the group receiving 150 mg twice a day than in the placebo group) and increases in levels of liver aminotransferases were more frequent in the group receiving 150 mg of BIBF 1120 twice daily than in the placebo group. In patients with idiopathic pulmonary fibrosis, BIBF 1120 at a dose of 150 mg twice daily, as compared with placebo, was associated with a trend toward a reduction in the decline in lung function, with fewer acute exacerbations and preserved quality of life. (Funded by Boehringer Ingelheim; ClinicalTrials.gov number, NCT00514683 .).

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Pirfenidone in idiopathic pulmonary fibrosis.

H Taniguchi, M Ebina, Y Kondoh … (2010)

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease without proven effective therapy. A multicentre, double-blind, placebo-controlled, randomised phase III clinical trial was conducted in Japanese patients with well-defined IPF to determine the efficacy and safety of pirfenidone, a novel antifibrotic oral agent, over 52 weeks. Of 275 patients randomised (high-dose, 1,800 mg x day(-1); low-dose, 1,200 mg x day(-1); or placebo groups in the ratio 2:1:2), 267 patients were evaluated for the efficacy of pirfenidone. Prior to unblinding, the primary end-point was revised; the change in vital capacity (VC) was assessed at week 52. Secondary end-points included the progression-free survival (PFS) time. Significant differences were observed in VC decline (primary end-point) between the placebo group (-0.16 L) and the high-dose group (-0.09 L) (p = 0.0416); differences between the two groups (p = 0.0280) were also observed in the PFS (the secondary end-point). Although photosensitivity, a well-established side-effect of pirfenidone, was the major adverse event in this study, it was mild in severity in most of the patients. Pirfenidone was relatively well tolerated in patients with IPF. Treatment with pirfenidone may decrease the rate of decline in VC and may increase the PFS time over 52 weeks. Additional studies are needed to confirm these findings.

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Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome.

J. W. Song, S-B. Hong, C.-M. Lim … (2011)

Although acute exacerbation of idiopathic pulmonary fibrosis (IPF) has become well recognised, the reported incidence and outcomes are highly variable, and risk factors are unknown. The aim of this study was to estimate the incidence, risk factors and impact of acute exacerbations, and other known causes of rapid deterioration. This was a retrospective review of 461 patients with IPF (269 cases were biopsy-proven). The median follow-up period was 22.9 months. Rapid deterioration requiring hospitalisation occurred in 163 (35.4%) patients, with multiple episodes in 42 patients. Acute exacerbation was the most frequent cause (55.2%), followed by infection. The 1- and 3-yr incidences of acute exacerbation were 14.2 and 20.7%, respectively. Never having smoked and low forced vital capacity (FVC) were significant risk factors. The in-hospital mortality rate was 50.0%, and the 1- and 5-yr survival rates from the initial diagnosis were 56.2 and 18.4%, respectively. Acute exacerbation was a significant predictor of poor survival after the initial diagnosis, along with increased age, low FVC and diffusing capacity of the lung for carbon monoxide, and steroid use with or without cytotoxic therapy. 1- and 3-yr incidences of acute exacerbation were 14.2 and 20.7%, respectively. Never having smoked and low FVC were risk factors. Acute exacerbation had a serious impact on the overall survival of the patients with IPF.

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Author and article information

Journal

Journal ID (nlm-ta): Interstitial Lung Disease

Title: Interstitial Lung Disease

Publication date PMC-release: 19 May 2017

Publication date (Print): 2018

Publication date (Electronic): 19 May 2017

Pages: 131-139

Article

Publisher ID: B978-0-323-48024-6.00011-2

DOI: 10.1016/B978-0-323-48024-6.00011-2

PMC ID: 7151865

SO-VID: 353a63b2-889d-4fc5-9f6d-16bb33287012

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Acute Exacerbations in Patients With Idiopathic Pulmonary Fibrosis

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Hematology and Oncology Discovery

Most cited references 85

Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis.

Pirfenidone in idiopathic pulmonary fibrosis.

Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome.

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