Predictors of Acute Exacerbation in Biopsy-proven Idiopathic Pulmonary Fibrosis

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  • Predictors of AE in biopsy-proven IPF

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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.

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