oa Current Allergy & Clinical Immunology - Common variable immunodeficiency in South Africa : primary immunodeficiency disorders

Volume 28, Issue 3
  • ISSN : 1609-3607



Common variable immunodeficiency (CVID) is the commonest symptomatic primary antibody deficiency in both adults and children. It is underdiagnosed in South Africa (SA) with no registered cases in many provinces. CVID is a clinically heterogeneous disorder with evolving diagnostic criteria. However, recurrent infections related to humeral immune system failure and hypogammaglobulinemia remain the cornerstones of diagnosis with straightforward initial investigations. In developing countries with limited laboratory infrastructure and decentralised healthcare, ongoing research into cheap screening strategies to detect low antibody levels and education to raise clinician awareness are critical to improved care. The increased availability of genetic testing globally has led to the discovery that up to 20% of patients with CVID or CVID-like disease have single gene defects. Access to genetic diagnosis should also increase in SA over the coming years.

Principles of management involve the prevention of infections, together with screening and prompt treatment of the non-infectious complications of immune dysregulation such as autoimmunity, lymphocytic hyperplasia and enteropathy. A multidisciplinary team of specialists optimally delivers care. Immunoglobulin replacement therapy (IRT) is the mainstay of treatment an poses an number of specific challenges in the SA context. Access, product availability and cost of therapy are important issues, while there is very limited infrastructure for adequate patient training and long-term support of therapy. Advocacy by immunologists, patients and patient groups is required if the particular needs of CVID patients are to be met within the many competing healthcare demands of SA.

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