ABSTRACT
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Subject(s)
Adult , Humans , Male , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Antibodies, Antineutrophil Cytoplasmic/blood , Biomarkers/blood , Biopsy , Drug Therapy, Combination , Fluorescent Antibody Technique , Glomerulonephritis, IGA/complications , Immunosuppressive Agents/therapeutic use , Myeloblastin/immunology , Treatment OutcomeABSTRACT
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