22–29 April 2021 is World Primary Immunodeficiency Week. Primary immunodeficiencies (PIs or PIDs) are caused by hereditary gene mutations that result in the disruption of normal immune function. There are over 380 different chronic PID disorders, ranging widely in severity. Severe combined immune deficiencies (SCIDs), which are characterised by the disturbed development of T and B cells, are the most severe form of PID. Although rare, the condition can be life-threatening.
Around 6 million people worldwide are thought to live with primary immunodeficiencies, with 70–90% of these cases still undiagnosed. Current therapies for several PIDs include immunoglobulin therapies, haematopoietic stem cell transplantation, gene therapy and in the case of infection, aggressive use of antibiotics, antifungals and interferon to stimulate immune system function.
To call attention to World Primary Immunodeficiency Week, our official journal Clinical & Experimental Immunology has created this Virtual Issue highlighting some of our most impactful primary immunodeficiency research from the last three years. This collection features cutting-edge reviews and original research, focusing on topics including PID diagnosis and pathogenesis in chronic granulomatous disease, Behçet’s disease and other PIDs, as well as clinical guidelines on the management of non-infectious CVID complications from the UK Primary Immunodeficiency Network.
We hope you enjoy reading this collection of articles and support World Primary Immunodeficiency Week by spreading awareness on social media using the hashtag #WorldPIWeek. If you share these articles, please loop in @CEIjournal!
Clinical & Experimental Immunology is an official journal of the British Society for Immunology. Profits derived from the sale of the journal are invested back into the BSI, providing major financial support for the Society's activities.
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