Society guideline links: Inborn errors of immunity (previously called primary immunodeficiencies)

Introduction — 

This topic includes links to society and government-sponsored guidelines from selected countries and regions around the world. We will update these links periodically; newer versions of some guidelines may be available on each society's website. Some societies may require users to log in to access their guidelines.

The recommendations in the following guidelines may vary from those that appear in UpToDate topic reviews. Readers who are looking for UpToDate topic reviews should use the UpToDate search box to find the relevant content.

International

●International Union of Immunological Societies (IUIS): Human inborn errors of immunity, update on the classification (2024)

●Clinical practice guidelines for the immunological management of chromosome 22q11.2 deletion syndrome and other defects in thymic development (2023)

●Updated management guidelines for adenosine deaminase deficiency (2023)

●IUIS: Phenotypical classification for human inborn errors of immunity, update (2022)

Canada

●Canadian Paediatric Society (CPS): Practice point on immunization of the immunocompromised child – Key principles (2018, reaffirmed 2024)

●CPS: Practice point on safe living strategies for the immunocompromised child (2019, reaffirmed 2024)

●Choosing Wisely Canada: Don't use immunoglobulin therapy for recurrent infections unless impaired antibody responses to vaccines are demonstrated (2023)

United States

●American Academy of Allergy, Asthma & Immunology (AAAAI): Diagnostic interpretation of genetic studies in patients with primary immunodeficiency diseases – A working group report (2020)

●Immune Deficiency Foundation (IDF): Patient & family handbook for primary immunodeficiency diseases, sixth edition (2019)

●AAAAI, American College of Allergy, Asthma & Immunology (ACAAI), and Joint Councilof Allergy, Asthma & Immunology (JCAAI): Practice parameter for the diagnosis and management of primary immunodeficiency (2015)

●IDF: Diagnostic & clinical care guidelines for primary immunodeficiency diseases, third edition (2015)

Europe

●European Society for Immune Deficiencies (ESID): Guidelines for the management of patients with congenital athymia (2024)

●European Society for Blood and Marrow Transplantation (EBMT)/ESID: Guidelines for hematopoietic stem cell transplantation for inborn errors of immunity (2021)

United Kingdom

●British Society for Immunology (BSI)/United Kingdom Primary Immunodeficiency Network: Consensus statement on managing non‐infectious complications of common variable immunodeficiency disorders (2019)

Australia–New Zealand

●Australasian Society of Clinical Immunology and Allergy (ASCIA): Inborn errors of immunity (primary immunodeficiencies) clinical care standard (2024)

●ASCIA: Intravenous immunoglobulin (IVIg) replacement therapy infusion rates and adverse reactions guide (2024)

●ASCIA: Newborn screening for severe combined immune deficiency (SCID) and BCG vaccination position statement (2024)

●ASCIA: Position statement – Immunoglobulin replacement therapy (IRT) for primary immunodeficiency (PID) (2024)

●ASCIA: Guideline for the diagnosis, management and transplantation of severe combined immunodeficiency (SCID) in Australia and New Zealand (2019)

●ASCIA: Position statement – Subcutaneous immunoglobulin (SCIg) (2018)

●ASCIA: Primary immunodeficiencies (PID) clinical update (2017)