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Immunodeficiency and Autoimmunity

ImmunodeficienciesInborn 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 diseasesAutoimmune 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 Inborn Errors of Immunity (Primary Immunodeficiencies) Clinical Care Standard

ASCIA Consensus Recommendations: Vaccination with Shingrix to Prevent Shingles for Immunosuppressive Conditions and Therapies - Content currently being finalised

ASCIA Immunodeficiency e-training courses - PID, IRT, HAE

ASCIA Immunodeficiency Strategy for Australia and New Zealand 

ASCIA Newborn Screening for Severe Combined Immune Deficiency (SCID) and BCG Vaccination Position Statement 

ASCIA Diagnosis and Management of Severe Combined Immunodeficiency (SCID) in Australia and New Zealand: A TAPID Consensus Guideline

ASCIA PID Clinical Update 

References - Immunodeficiency

ASCIA ID Register How to access information (available to ASCIA members only)

Immunoglobulin Replacement Therapy (IRT) 

ASCIA Position Statement - IRT in PID 

ASCIA Guidelines - Standardised IVIg Infusion Rates for IRT - Document currently under review

ASCIA Treatment Summary for Patients on Immunoglobulin Therapy

Subcutaneous Immunoglobulin (SCIg)

ASCIA SCIg Checklists - Nurse Competency and Patient Training 

ASCIA SCIg Treatment Plan

ASCIA SCIg Travel Plan 

ASCIA SCIg Position Statement  

ASCIA Guide – Setting up a SCIg program in a hospital

Hereditary Angioedema (HAE)

ASCIA HAE Position Paper and Management Plan 

References - HAE

ASCIA Webinar - 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 November 2024