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Autoimmunity in patients with primary immunodeficiency

Markus G Seidel, MD
Section Editor
E Richard Stiehm, MD
Deputy Editors
Anna M Feldweg, MD
Monica Ramirez Curtis, MD, MPH


Patients with primary immunodeficiency (PID) have dysregulated immune processes, which can result in an increased susceptibility to infectious diseases, autoimmune disorders, and malignancies.

This topic will discuss the most common manifestations of autoimmunity in patients with PID and review the autoimmune disorders associated with the most prevalent PID disorders. Malignancies in patients with PID are reviewed separately. (See "Malignancy in primary immunodeficiency".)


PIDs are classified into nine major categories of disorders [1]:

Combined immunodeficiencies without syndromic features. (See "Combined immunodeficiencies" and "Severe combined immunodeficiency (SCID): An overview".)

Combined immunodeficiencies with syndromic features. (See "Syndromic immunodeficiencies".)

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Literature review current through: Nov 2017. | This topic last updated: Dec 11, 2017.
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