@article{oai:hama-med.repo.nii.ac.jp:00003736,
 author = {Kishaba, Tomoo and Hozumi, Hironao and Fujisawa, Tomoyuki and Nei, Yuichiro and Enomoto, Noriyuki and Sugiura, Hiroaki and Kitani, Masashi and Suda, Takafumi},
 issue = {3},
 journal = {Respiratory Investigation},
 month = {May},
 note = {Background: Acute exacerbation (AE) is a major cause of death in patients with idiopathic pulmonary fibrosis (IPF). Current evidence on AE-IPF was largely based on clinical, rather than pathological, analyses.
Methods: We investigated AE incidence and its predictors using clinical, radiological, and pathological data of patients diagnosed with IPF by multi-disciplinary discussion.
This study, a secondary analysis of previous research, included 155 patients with IPF who underwent surgical lung biopsy (SLB). Cumulative AE incidence was evaluated by the Kaplan?Meier method.
Predictors of AE-IPF were analyzed with a Fine-Gray subdistribution hazard model. Sub-analysis was performed using propensity score-matching analysis.
Results: In this cohort, median age was 66 years and median percent-predicted forced vital capacity 82.8%. The cumulative AE incidence rates at 30-days and one-year post-SLB were 1.9% and 7.6%, respectively. On multivariable analysis, a lower percent-predicted diffusing capacity of the lung for carbon monoxide (%DLCO) (hazard ratio 0.98 per 1% increase, P=0.02) and fibroblastic foci (FF)-present (vs. absent; hazard ratio 3.01, P=0.04) were independently associated with higher incidence of AE. The propensity score-matching analysis with adjustment for age, gender, and %DLCO revealed that the cumulative AE incidence rate was significantly higher in the FF-present subgroup than in the FF-absent subgroup (1-year incidence rate, 10.5% vs. 0%, respectively; P=0.04 by Gray’s test).
Conclusions: FF and %DLCO were independent predictors of AE in patients with biopsy-proven IPF. FF may be associated with the pathogenesis of AE-IPF.},
 pages = {177--184},
 title = {Predictors of Acute Exacerbation in Biopsy-proven Idiopathic Pulmonary Fibrosis},
 volume = {58},
 year = {2020}
}