Physicians may be unaware of the clinical manifestations of autoimmune disease among patients with known primary immunodeficiencies.

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Autoimmunity results from the production of functional and pathogenic autoantibodies against target organs. The subsequent inflammation, degeneration, tissue injury, and end-organ failure in the setting of primary immunodeficiency (PID) exacerbates the course of PID. Multiple systems may be affected, including vascular, joint, skin, endocrine, muscular, and circulating blood cells. Preliminary screening for signs of autoimmune disease in the patient with PID can include hemolytic anemia, markers for inflammation, and the presence of circulating autoantibodies (eg, rheumatoid factor and antinuclear antibody). The connection between immunodeficiency and autoimmunity is well recognized among pediatric immune disorders. Autoimmune manifestations represent the second most common clinical consequence of PID; recurrent infections remain the most common. Advances in understanding the molecular and cellular biology of disease have led to...

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