The use of databases in primary immunodeficiencies

Abstract

Purpose of review: We provide an overview on the latest developments in primary immunodeficiency registries worldwide, on the basis of the recent literature amended by some older references to achieve completeness.

Recent findings: New primary immunodeficiency registries are emerging worldwide, although existing databases continue to thrive and provide valuable insights for clinicians and researchers.

Summary: In the area of rare disease research, data on a meaningful number of patients can only be achieved via collaboration. Registries for primary immunodeficiency are organized on different geographic levels and appear in various technical forms. Some registries are operated within single departments or hospitals, whereas others collect data from a country in the form of a national registry. With modern information technology and networks, it has become feasible to easily extend documentation to the transnational level. Most patient registries cover similar but not identical sets of data, whereas some have a special focus on, for example, genetics or incorporate only data from patients who have undergone a specific form of treatment. This review shows the usefulness and power of international immunodeficiency registries, as well as possible hurdles and limitations.