Open AccessA hepatitis C-positive patient with new onset of nephrotic syndrome and systemic amyloidosis secondary to common variable immunodeficiency
Author(s) -
Kültiğin Türkmen,
M.H. Anil,
Yalçın Solak,
Hüseyin Atalay,
Hasan Esen,
Halil Zeki Tonbul
Publication year - 2010
Publication title -
annals of saudi medicine/annals of saudi medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.373
H-Index - 44
eISSN - 0975-4466
pISSN - 0256-4947
DOI - 10.4103/0256-4947.67085
Subject(s) - medicine , nephrotic syndrome , amyloidosis , common variable immunodeficiency , immunodeficiency , pediatrics , dermatology , immunology , antibody , immune system
Common variable immunodeficiency (CVID) is a heterogenous group of predominantly antibody-deficiency disorders that make up the greatest proportion of patients with symptomatic primary hypogammaglobulinemia. The rare coincidence of amyloidosis and hypogammaglobulinemia has been reported previously. Contrary to the usual insidious, slowly progressive disease following hepatitis C infection, a rapidly progressive cirrhotic form can develop in hypogammaglobulinemic patients. We report a HCV-positive patient with a new onset of nephrotic syndrome and systemic amyloidosis secondary to CVID. Blood analyses showed serum creatinine of 1.8 mg/dL and serum albumin of 3.1 gm/dL; 24-h urinary protein was 11 800 mg/day. Serum immunoglobulin levels were IgG 340 mg/dL, IgM 18 mg/dL, IgA 11 mg/dL. Duodenal biopsy revealed AA-type amyloidosis with potassium permanganate and Congo red staining. After a month of antiproteinuric therapy, the proteinuria was reduced to 3350 mg/day.