OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis for which there are few comprehensive real-life data. This French study is the first to assess the epidemiology and the clinical and economic burden of EGPA on the SNDS.

METHODS: EGPA patients were identified by a long-term disease with an ICD-10 code M30 (polyarteritis nodosa and related conditions) or M301 (polyarteritis with lung involvement [Churg-Strauss]) and/or a hospitalization with a code M301. As the M30 code is not specific, patients hospitalized for another vasculitis were excluded. Study period ran from 2010 to 2019. Incident 2012-2016 patients were followed up to 2019 to assess disease burden. EGPA relapse was defined as a doubling of corticosteroid dose, a new treatment initiation, or an EGPA hospitalization.

RESULTS: In 2019, 5,733 EGPA patients were identified, including 232 new cases, corresponding to a prevalence (incidence) of 85.3 (3.5) cases per million. Among 2012-2016 incident cases,1-year and 5-year EGPA relapse rates were 53.8% (51.3%-56.2%) and 69.3% (66.9%-71.6%) respectively; with a median EGPA relapse-free survival of 9.7 months. Reimbursed overall costs were highest in the first year after index date, with a median (Q1-Q3) of 12,261€ (3,188€-31,809€), dropped to 3,081€ (1,096€-11,178€) in the second year and continued to decrease to 1,781€ (467€-6,865€) in the fifth year. All-cause hospitalizations were the main cost drivers representing 82.0% of cost during the first year, and 62%-64% afterward.

CONCLUSIONS: This SNDS study assesses the epidemiology of EGPA in the overall French population. It provides recent data on the clinical burden, characterized by frequent and rapid relapses, and highlights the economic burden, with high costs during the first year. Matching the SNDS with the Groupe Français d’Etude des Vascularites (GFEV) registry will provide additional clinical data and the opportunity to refine the identification of EGPA in the SNDS.