The French national registry of primary immunodeficiency diseases

Abstract

The French National Reference Center of Primary Immunodeficiencies (CEREDIH) was established in 2005 and now constitutes a nationwide network of pediatric and adult medicine departments in university medical centers. The registry comprises a total of 3,083 patients (mainly children), with an overall prevalence of 4.4 cases per 100,000 inhabitants. Predominantly B-cell immunodeficiencies are the most common diseases observed (43%). The proportion of common variable immunodeficiencies (CVIDs, 14%) is lower than reported by national registries in other developed countries. The data suggest that although referral to expert centers is fairly adequate for children, this is not yet the case in France for adults. The distribution of primary immunodeficiencies (PIDs) varied significantly across distinct geographical areas and this suggested regional differences in patient care. As the world's largest national registry of PIDs, CEREDIH provides a basis for both further studies and activities aimed at raising the physicians' awareness of PIDs (notably in adults).