Prediction of Relapse and Glucocorticoid Dependence in Eosinophilic Granulomatosis With Polyangiitis: Findings From a Large European Cohort.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by eosinophilia, asthma, and ear, nose, and throat (ENT) involvement. Although glucocorticoids (GCs) are effective in controlling symptoms, relapses and GC dependence are common. The aim of this study was to develop predictive models for vasculitis relapse and GC-dependent asthma and/or ENT symptoms. This multicenter European retrospective cohort study included patients with EGPA fulfilling the 2022 American College of Rheumatology/EULAR criteria. Using Fine-Gray and logistic regression, we developed two multivariable prediction models, one for vasculitis relapse and another for GC-dependent asthma and/or ENT symptoms at 2 Internal validation was performed using bootstrapping. A total of 809 patients were observed for a median of 72 months (interquartile range 37-115). Vasculitis relapse occurred in 228 patients with a 12-year cumulative incidence of 41.2% (95% confidence interval 36.3-46.8). GC-dependent asthma and/or ENT symptoms were observed in 66.4% at two years. Predictors of vasculitis relapse included age (nonlinear), GC-dependent asthma before EGPA diagnosis (hazard ratio [HR] 1.57), arthralgia (HR 1.27), myocarditis (HR 1.74), peripheral neuropathy (HR 1.39), myeloperoxidase-antineutrophil cytoplasmic antibody (HR 1.56), and baseline eosinophil count (nonlinear). Predictors of GC-dependent asthma and/or ENT symptoms included older age (odds ratio [OR] 0.98 per year), GC-dependent asthma at diagnosis (OR 1.50), chronic sinusitis (OR 1.78), and baseline eosinophil count (OR 0.70 per 10⁹/L). Using a large cohort with EGPA, we developed predictive models for vasculitis relapse and GC-dependent asthma and/or ENT symptoms. These tools may help guide treatment decisions. Prospective external validation in the current therapeutic era is warranted.