Baseline High-Resolution CT Findings Predict Acute Exacerbation of Idiopathic Pulmonary Fibrosis: German and Japanese Cohort Study.

Nobuaki Shime,Noboru Hattori,Hiroshi Iwamoto,Hilmar Kühl,Chihiro Hirano,Josune Guzman,Shinichiro Ohshimo,Nobuoki Kohno,Daisuke Komoto,Ulrich Costabel,Yasushi Horimasu,Kazuo Awai,Hironobu Hamada,Francesco Bonella,Kazunori Fujitaka

doi:10.3390/jcm8122069

Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major cause of morbidity and death in IPF. However, sensitive predictive factors of AE-IPF have not been well-investigated. To investigate whether high-resolution computed tomographic (HRCT) abnormalities predict AE-IPF in independent ethnic cohorts, this study included 121 patients with IPF (54 German and 67 Japanese; mean age, 68.5 ± 7.6 years). Two radiologists independently visually assessed the presence and extent of lung abnormalities in each patient. Twenty-two (18.2%) patients experienced AE-IPF during the follow-up. The incidence of AE-IPF was significantly higher in the Japanese patients (n = 18, 26.9%) than in the German patients (n = 4, 7.3%, p < 0.01). In the Kaplan–Meier analysis, patients with a larger extent of ground glass opacity (GGO), fibrosis, and traction bronchiectasis experienced an earlier onset of AE-IPF (p = 0.0033, 0.0088, and 0.049, respectively). In the multivariate analysis, a larger extent of GGO and fibrosis on HRCT were independent predictors of AE-IPF (p = 0.026 and 0.037, respectively). Additionally, Japanese ethnicity was independently associated with the incidence of AE-IPF after adjustment for HRCT findings (p = 0.0074). In conclusion, a larger extent of GGO and fibrosis on HRCT and Japanese ethnicity appear to be risk factors for AE-IPF.

Highlights

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis of the lung with unknown etiology resulting in a continuous deterioration of pulmonary function and poor outcomes
We investigated whether High-resolution computed tomography (HRCT) abnormalities could predict the occurrence of acute exacerbation of IPF (AE-IPF) in the independent two ethnic cohorts by using the same definition criteria of AE-IPF, and demonstrated that a larger extent of ground glass opacity (GGO) and fibrosis area on HRCT and Japanese ethnicity were independent risk factors for AE-IPF
The recent statements on IPF emphasized that the discrimination of “pure” GGO from GGO associated with traction bronchiectasis or other features of fibrosis was very important for identifying IPF and AE-IPF [23]

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive fibrosis of the lung with unknown etiology resulting in a continuous deterioration of pulmonary function and poor outcomes. Some patients experience abrupt deterioration in their respiratory status with no identifiable cause, which is termed acute exacerbation of IPF (AE-IPF). There have been several studies that identified clinical risk factors for AE-IPF, including poor pulmonary function [3,4,5], rapid decline in vital capacity (VC) [6,7,8], and pulmonary hypertension [9]. Right-heart catheterization for confirming pulmonary hypertension is invasive. Several biomarkers, such as KL-6, Surfactant protein-D, matrix metalloproteinases, and CC Chemokine Ligand-18 have been investigated as predictors of AE-IPF, but they have not yet been validated [10]. The association between HRCT characteristics and the risk of AE-IPF has not been extensively explored

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