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Granulomatous disease in selective IgA deficiency

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Although common variable immunodeficiency (CVID) is sometimes associated with sarcoidosis/granulomatous disease, there have only been isolated reports of selective immunoglobulin A (IgA) deficiency and granulomatous disease. We present a patient with IgA deficiency who developed Heerfordt syndrome, a variant of neurosarcoidosis. This specific entity has not been previously reported to occur in IgA deficiency. Our case expands the reported associations of IgA deficiency and provides another example to the paucity of reported cases of sarcoidosis occurring in patients with IgA deficiency. As CVID and IgA deficiency have common underlying genetic factors, such an association is biologically plausible.

Keywords: Heerfordt syndrome; IgA deficiency; common variable immunodeficiency; granuloma

Document Type: Research Article


Affiliations: 1: Rheumatology Department, Royal Adelaide Hospital and Department of Medicine, University of Adelaide, 2: Repatriation General Hospital, 3: The Queen Elizabeth Hospital,

Publication date: September 1, 2007

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