Immunodeficiency and Autoimmunity
Inborn errors of immunity (IEI) include primary immune deficiencies (PID) and are a group of more than 480 potentially serious chronic medical conditions. They are caused by defects in genes that control the immune system and can lead to frequent or severe infections, and other chronic immune system disorders, including autoimmune problems.
ASCIA Immunoglobulin Replacement Therapy (IRT) and Subcutaneous Immunoglobulin (SCIg) information is mostly not specific for IEI/PID, and can be used by other medical specialties.
The International Patient Organisation for PIDs (IPOPI) has released an educational video series on PIDs, to increase the understanding of early detection and treatments for these conditions.
Autoimmune diseases are a broad range of more than 80 related disorders that include rheumatoid arthritis, systemic lupus erythematosus (lupus) and vasculitis disorders (inflammation of blood vessels). They are thought to be inherited in many cases, but factors such as infections and some drugs (medications) may also play a role in triggering autoimmune diseases.
Autoimmune diseases are usually diagnosed using a combination of clinical history, blood tests and other investigations such as x-rays and a biopsy. In autoimmune diseases, the immune system attacks the body's own cells, tissues and organs, resulting in inflammation and damage, so treatments aim to relieve symptoms, minimise organ and tissue damage and preserve organ function.
Information for Health Professionals
ASCIA Clinical Care Standard - Inborn Errors of Immunity (Primary Immunodeficiencies)
ASCIA Immunodeficiency e-training courses - PID, IRT, HAE
ASCIA Immunodeficiency Strategy for Australia and New Zealand
ASCIA References - Immunodeficiency
ASCIA ID Register How to access information (available to ASCIA members only)
Immunoglobulin Replacement Therapy (IRT): Intravenous Immunoglobulin (IVIg) and Subcutaneous Immunoglobulin (SCIg)
ASCIA Position Statement - IRT in PID
ASCIA Position Statement - SCIg
ASCIA Guidelines - IVIg Replacement Therapy Infusion Rates and Adverse Reactions Guide
ASCIA Treatment Summary - Patients on Immunoglobulin Therapy
ASCIA Checklists - SCIg Nurse Competency and Patient Training
ASCIA Guide – Setting up a SCIg program in a hospital
Hereditary Angioedema (HAE)
ASCIA Position Paper and Management Plan - HAE
Auotimmunity
ASCIA Position Paper - Laboratory Tests for Autoimmune Diseases
Information about Vasculitis is available at www.anzvasculitis.org/medical-professionals/
Further Information
Webpage updated January 2025