The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data.

Background: Common variable immunodeficiency disorders (CVID) are a group of rare innate disorders characterized by specific antibody deficiency and increased rates of infections, comorbidities and mortality. The burden of CVID in Europe has not been previously estimated. We performed a retrospective analysis of the European Society for Immunodeficiencies (ESID) registry data on the subset of patients classified by their immunologist as CVID and treated between 2004 and 2014.

[The ESID Online Database for primary immunodeficiencies. First analyses with regard to Germany and Europe].

Background And Methods: Over the past 3 years, with support by the European Union within the 6th Framework Programme and by the pharmaceutical industry, the ESID Online Database has collected data from 7,047 patients with primary immunodeficiencies (PIDs) in 30 countries. The system is available via a standard internet browser for users in registered documenting centers with their personal login. It comprises a common core dataset for 212 PIDs and large disease-specific data models for 33 diseases.

Primary immunodeficiency registries.

Purpose Of Review: Research in the field of rare diseases such as primary immunodeficiencies can be significantly improved with sufficient patient numbers. Patient registries can help provide the basis for this by collecting data over a longer period of time and by connecting centres nationally or even internationally. The present article reviews recent publications both on database systems themselves and the research performed using results from these databases.