246. Predictive factors of eosinophilic granulomatosis with polyangiitis long term evolution: data from a European cohort
Creators: name: Papo1, Matthias
name: Martinot2, Pauline
name: Sinico3, Renato A.
name: Vitor Teixeira4, 5
name: Nils Venhoff6,
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) effectively control the disease, but relapses and/or GC-dependence 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. <strong>Methods:</strong> We set up a multicenter European retrospective study including 809 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. <strong>Results:</strong> Mean 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% (CI95% 36-47). At two years of follow-up, 66,4% of patients had GC-dependent asthma and/or ENT manifestations. A model predicting vasculitis relapse included the following variables: older age at EGPA diagnosis (HR 1.07 [IC 95% 0.98-1.18]), GC-dependent asthma before EGPA diagnosis (HR 1.43 [IC 95% 1.16-1.77]), arthralgia (HR 1.32 [IC 95% 1.09-1.61]), myocarditis (HR 1.27 [IC 95% 0.96-1.69]), peripheral neuropathy (HR 1.33 [IC 95% 1.09-1.63]), positive MPO-ANCA (HR 1.37 [IC 95% 1.10-1.72]), C-reactive protein level (HR 1.07 [IC 95% 1.00-1.15]) and lower eosinophils count (HR 0,76 [IC 95% 0,67-0,86]) at baseline. The final model allowed the development of a nomogram and the calculation of a score predicting the vasculitis relapse risk at 5 and 12 years of follow-up. A model predicting GC-dependent asthma and/or ENT manifestations at two years of follow-up included the following variables: younger age at EGPA diagnosis (OR 0.98 [IC 95% 0.97-0.99]), history of chronic sinusitis before EGPA diagnosis (OR 1.11 [IC 95% 0.70-1.77]), ENT manifestations at baseline (OR 1.65 [IC 95% 1.09-2.47]), peripheral neuropathy (OR 1.36 [IC 95% 1.01-1.84]), the absence of MPO-ANCA (OR 0.68 [IC 95% 0.51-0.92]) and lower eosinophils count (HR 0.45 [IC 95% 0.28-0.72]). The final model allowed the development of nomogram and the calculation of a score predicting GC-dependent asthma and/or ENT manifestations at 2 years. <strong>Conclusion:</strong> Through a large retrospective European EGPA cohort, we developed two models for the prediction of vasculitis relapse during follow-up and GC-dependent asthma and/or ENT manifestations at 2 years. These models should be validated in prospective studies, and could be used to guide the use of biotherapies targeting B cells or eosinophils. <br> <strong>Disclosures:</strong> nothing to disclose.