A hepatitis C-positive patient with new onset of nephrotic syndrome and systemic amyloidosis secondary to common variable immunodeficiency.
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.
Turkmen
Kultigin
K
Selcuk University Meram School of Medicine, Nephrology Department, Konya, Turkey. mdkt2010@yahoo.com
Anil
Melih
M
Solak
Yalcin
Y
Atalay
Huseyin
H
Esen
Hasan
H
Tonbul
Halil Z
HZ
D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications; D000686:Amyloidosis / Q000188:drug therapy / Q000209:etiology*; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000890:Anti-Infective Agents / Q000627:therapeutic use; D002939:Ciprofloxacin / Q000627:therapeutic use; D017074:Common Variable Immunodeficiency / Q000150:complications*; D005873:Giardiasis / Q000150:complications / Q000188:drug therapy; D006526:Hepatitis C / Q000150:complications; D018937:Hepatitis C Antibodies / Q000097:blood*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D019808:Losartan / Q000627:therapeutic use; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology*; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D017257:Ramipril / Q000627:therapeutic use
D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D000890:Anti-Infective Agents; D018937:Hepatitis C Antibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D002939:Ciprofloxacin; D019808:Losartan; D017257:Ramipril