Classification of primary immunodeficiency disorders: One-fits-all does not help anymore

摘要

Primary immunodeficiency diseases (PIDs) include a heterogeneous group of conditions that affect development and/or function of the immune system. Most forms of PIDs are inherited as Mendelian traits. In the last years, advances in genetics and cell biology have permitted to define the molecular basis and the pathophysiology of several new forms of PIDs. These achievements are reflected in the latest classification of PIDs that has been produced by the International Union of Immunological Societies (IUIS) Expert Committee on Primary Immunodeficiency . By providing essential information on the clinical and immunological features of the PIDs, such classification is meant to facilitate the diagnostic approach to these disorders.