Renal involvement in a syndrome of vasculitis complicating HBsAg negative cirrhosis of the liver.
Six HBsAg negative patients with cirrhosis of the liver (CL) presented with recurrent bouts of palpable purpura in the legs due to small vessel leucocytoclastic vasculitis. In addition, all patients had renal failure, proteinuria and microhaematuria. Renal biopsy disclosed either diffuse proliferative (3 cases) or focal necrotising glomerulonephritis with crescents (2 cases). One patient had IgM-IgG mixed cryoglobulinaemia (type II). Four patients died of complications of their CL. Hepatocellular carcinoma was found in 1 case. In the patient without renal biopsy renal function improved following steroids and cyclophosphamide. The pathogenesis of this syndrome of cutaneous vasculitis with severe glomerular involvement in CL is unknown but could be immune-complex mediated.
D000368:Aged; D005260:Female; D005921:Glomerulonephritis / Q000150:complications*; D006514:Hepatitis B Surface Antigens; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications*
D006514:Hepatitis B Surface Antigens