Granulomatous renal disease in a patient with common variable immunodeficiency.
Common variable immunodeficiency (CVID), the most common cause of primary hypogammaglobulinemia, is characterized by a decreased serum immunoglobulin level, recurrent infections, and the occurrence of various autoimmune diseases. Granulomatous disease has been reported previously in several patients with CVID, with granuloma occurring in the lymph nodes, spleen, liver, central nervous system, and bone marrow. We report the first published case of renal granulomatous disease in a CVID patient presenting with subacute renal failure. Renal function partially recovered after corticosteroid treatment and intravenous immunoglobulin infusions. The pathogenesis of granulomatous disease in CVID is unclear but may involve monocyte and T-cell abnormalities.
Fakhouri
F
F
Department of Nephrology and Pathology, Hôpital Necker, Paris, France. fakhouri@necker.fr
Robino
C
C
Lemaire
M
M
Droz
D
D
Noël
L H
LH
Knebelmann
B
B
Lesavre
P
P
D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000209:etiology; D001706:Biopsy; D017074:Common Variable Immunodeficiency / Q000150:complications*; D005260:Female; D006099:Granuloma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology*