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Vol 23. no 1
March 2018

Rheumatologic manifestations of primary immunodeficiency diseases (summary)
Marcin Ziętkiewicz, Zbigniew Zdrojewski
Primary immunodeficiency (PID) constitutes the group of over 300 disorders of varied clinical picture. It is estimated that there might be approximately six million people suffering from PID worldwide. In the course of primary immunodeficiency not only recurring infections may occur, but also autoimmune disorders. This group also includes a number of diseases which are of interest for rheumatologists, such as connective tissue diseases (e.g. systemic lupus erythematosus). Moreover, the increased proneness to infections might be the cause of the occurrence of septic or reactive arthritis. In the course of several PID no recurring infections occur. In the light of the data published in 2017, based on the analysis of the fortunes of 2183 French patients suffering from primary immunodeficiency, rheumatic diseases might constitute the fourth largest group in terms of the disorders which are the most frequent in the course of PID. The only more frequent groups are autoimmune cytopenia, digestive system diseases and skin diseases (with the exception of vasculitis). The thesis presents the connections between the occurrence of systemic lupus erythematosus, arthritis, vasculitis and dermatomyositis, and the selected primary immunodeficiencies.
Keywords: primary immunodeficiency, autoimmunization, systemic lupus erythematosus, arthritis, vasculitis, dermatomyositis
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