Session Type: Poster Session A
Session Time: 1:00PM-3:00PM
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) frequently control the disease, but relapses and/or GC-dependency are frequent. Evolving concepts distinguish vasculitis-related symptoms from asthma and/or ENT manifestations. That distinction has become even more important since the development of B-cell and eosinophil-targeted therapies. Our objective was to build models to predict the evolution towards a relapse of vasculitis or towards a GC-dependent asthma and/or ENT manifestations.
Methods: We set up a multicenter European retrospective study of EGPA patients. EGPA diagnosis was made according to ACR and/or MIRRA criteria. We developed a multivariable prediction model using the PMSAMPSIZE algorithm: one model for the vasculitis relapse risk during follow-up (excluding asthma and/or ENT manifestations) and one model for the risk of GC-dependent asthma and/or ENT manifestations at two years of follow-up.
Results: We included 809 patients. Patients’ middle age was 52 (+/- 14.8) years, and median follow-up was of 72 months (IQR 37/115). During follow-up, 228 experienced at least one vasculitis relapse, with a cumulative incidence at 12 years of 41.2% (95% CI 36.3-46.8). At two years of follow-up, 66.4% of patients had GC-dependent asthma and/or ENT manifestations.
First, a model predicting the risk of vasculitis relapse included the following variables: age at diagnosis (nonlinear effect), GC-dependent asthma before EGPA diagnosis (HR 1.57 [1.24-2.55]), arthralgia (HR 1.27 [1.01-1.60]), myocarditis (HR 1.74 [1.16-2.62]), peripheral neuropathy (HR 1.39 [1.09-1.79]), MPO-ANCA (HR 1.56 [1.22-2.01]) and baseline eosinophils count (nonlinear effect]). Final model allowed to modelize a nomogram and calculate a score establishing the vasculitis relapse risk at 5 and 12 years of follow-up.
Second, a predicting the risk of GC-dependent asthma and/or ENT manifestations at 2 years included the following variables: age at diagnosis (per year, OR 0.98 [0.97-0.99]), GC-dependent asthma at EGPA diagnosis (OR 1.50 [1.02-2-20]), history of chronic sinusitis before EGPA diagnosis (OR 1.78 [1.27-2.48]), and baseline eosinophils count (per 10.109/L, OR 0.70 [0.49-1.00]). Final model allowed to modelize a nomogram and calculate a score establishing the risk of GC-dependent asthma and/or ENT manifestations at 2 years.
Conclusion: Through a large retrospective European EGPA cohort, we developed models predicting the vasculitis relapse risk and the risk of GC-dependent asthma and/or ENT manifestations at 2 years. These models must be validated in validation cohorts and could be used to guide the use of biotherapies targeting B cells or eosinophils.
To cite this abstract in AMA style:Papo M, Martinot P, Sinico R, Teixeira V, Venhoff N, Urban M, Mahrhold J, Locatelli F, Cassone G, Schiavon F, Seeliger B, Neumann T, Kroegel C, Groh M, Marvisi C, Samson M, Barba T, Jayne D, Troilo A, Thiel J, Hellmich B, Monti S, Montecucco C, Salvarani C, Kahn J, DUREL C, Mouthon L, Guillevin L, Emmi G, Vaglio A, Porcher R, Terrier B. Predictive Factors of Eosinophilic Granulomatosis with Polyangiitis Long-term Evolution: Data from a European Cohort [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/predictive-factors-of-eosinophilic-granulomatosis-with-polyangiitis-long-term-evolution-data-from-a-european-cohort/. Accessed .
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