Overview
- Santé publique France’s reanalysis of 2010–2021 records, drawing on Assurance Maladie data, finds stable incidence and mortality at about 2,500 diagnoses and 1,840 deaths per year.
- Localized incidence clusters are identified around Nîmes–Avignon–Alès (557 observed vs 449 expected), Guingamp–Lorient (520 vs 417), and Clermont-Ferrand (315 vs 241).
- Two excess-mortality zones are flagged near Lorient–Vannes (463 observed deaths vs 334 expected) and Saint-Étienne (339 vs 298).
- Regional variation is marked, with Brittany highest at roughly 3.77 cases per 100,000 and French Guiana lowest at 1.06, against a national range of 3–3.5 per 100,000.
- These rare diseases predominantly affect men around ages 70–79, progress rapidly without a curative treatment, and their causes remain unclear with gene–environment interactions under investigation.