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Primary Immune Deficiency

Primary Immunodeficiency Diseases (PIDs) & Inborn Errors of Immunity (IEIs)

Patients who suffer from infections that are more severe, more persistent, more unusual or occur more frequently may suffer from a Primary Immunodeficiency Disease (PID). PIDs form part of a bigger group of immune-related diseases that we now refer to as the Inborn Errors of Immunity or IEIs.

Cause of PIDs & IEIs

IEIs and PIDs result from variations in the genes that direct the immune functions. These variations may result in the loss of immune function, or loss of immune tolerance. They may even lead to gain in unwanted (more aggressive) immune responses. Patients then suffer from problematic infections. These infections may result from a specific type or different types of microbes. Other forms of immune dysfunction may precede or follow or be present at the same time. These include severe allergy, autoimmune diseases, exaggerated inflammation and even some cancers.

How many types of IEIs are there?

There are now more than 485 known IEIs. More are being identified with modern technology. Some of the PIDs may be individually rare but these diseases are collectively not rare. The aggregate contributes to a significant part of the health burden. We do not have exact prevalence figures for South Africa. International data show that about 1:1200 people suffer from a PID.

Be alert & test.

Many IEI & PID patients are only diagnosed after a long time of suffering. Sometimes when it is too late. They often receive repeated courses of antibiotics without knowing about the underlying fault in their immune system. There are many reasons to delayed diagnosis. Patients and health care workers are often not aware of the PIDs; or do not understand that these conditions may affect children and adults; or that they may present with more common infections that are often perceived as trivial; or that many patients who suffer from a PID may never need hospital admission; or that other problems like severe allergy or autoimmunity could be part of PID. Readily available tests can alert to the risk of an underlying PID.

Can we treat PIDs & IEIs?

PIDs are chronic conditions & patients deserve access to an early diagnosis and proper evidence-based treatment. Different forms of effective treatment are available. Some of these treatment options are affordable and others may be more expensive. Evidence and consensus-based guidelines are available to ensure fair access to resources.

Different forms of treatment are available. It is possible to improve quality of life, prevent permanent organ damage and save lives. Evolving targeted therapies and precision medicine offers much hope for many IEI patients.

Who is PIDDSA?

PIDDSA (Primary Immunodeficiency Diseases South Africa) is a medical professional working group of the Allergy Society of South Africa. PIDDSA assists doctors with IEI & PID articles, guidelines, dedicated training workshops and congress sessions. PIDDSA also supports the patient organisation for PID (PiNSA) and AFSA.

If you or your child have been diagnosed with a PID, or are experiencing severe, recurrent or unusual signs of infections, this site may be able to direct you to resources for help. We are not able to answer individual questions and requests.

Download our “Primary Immune Deficiency” leaflet for free