Fever of unknown origin in a patient with common variable immunodeficiency associated with multisystemic granulomatous disease

Non-caseating epithelioid granulomas have been described in a small number of patients with common variable immunodeficiency (CVID). We report a 26-year-old woman diagnosed with CVID nine years earlier, who developed non-caseating granulomas in the liver, bone marrow and skin. She was referred to our department for a fever of more than one year duration without apparent focus. Extensive search for underlying malignancy or occult infection was unremarkable. Empirical treatment with prednisone was begun and the patient showed a marked improvement. The literature on the association between CVID and non-caseating granulomatous disease, and the differential diagnosis of hepatic granulomas as a cause of fever of unknown origin, is also reviewed.