
This classification is the most up-to-date catalog of all known PIDs and acts as a current reference of the knowledge of these conditions and is an important aid for the molecular diagnosis of patients with these rare diseases. For each disorder, the key clinical and laboratory features are provided. In addition, we have added a table of acquired defects that are phenocopies of PIDs. In comparison to the previous version, more than 30 new gene defects are reported in this updated version. We report the updated classification of primary immunodeficiencies (PIDs) compiled by the Expert Committee of the International Union of Immunological Societies.

9Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia.8Meyer Children’s Hospital-Technion, Haifa, Israel.7Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY, USA.6Division of Immunology, Children’s Hospital Boston, Boston, MA, USA.5Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Imagine Institut, Necker Medical School, University Paris Descartes, Paris, France.Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA

3Clinical Immunology Unit, Casablanca Children’s Hospital, Ibn Rochd Medical School, King Hassan II University, Casablanca, Morocco.2Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait.1Department of Pediatrics, Kuwait University, Kuwait City, Kuwait.
