The spectrum of hepatobiliary disease in primary hypogammaglobulinaemia

Objective. To study the prevalence of hepatobiliary disease in a clinically and immunologically well-characterized group of 88 adult Norwegian patient s with primary hypogammaglobulinaemia. Subjects. Eighty-eight patients with primary hypogammaglobulinaemia were fo llowed and signs and symptoms of liver disease were recorded. The patients were examined clinically and radiologically on a regular basis with liver b iopsies performed when indicated. All patients were tested for hepatitis C virus (HCV) RNA, hepatitis G virus (HGV) RNA and hepatitis B virus (HBsAg). Results, Twenty-one patients were HCV RNA-positive, all having signs of chr onic liver disease. Only four patients were HGV RNA-positive, of whom two w ere also HCV RNA-positive. Amongst the 67 HCV RNA-negative patients, 26 had signs of chronic liver disease, including two who were HGV RNA-positive. H CV RNA-negative patients with liver disease had received intravenous immune globulin substitution more frequently, had a longer history of any form of immune globulin substitution and had a greater incidence of common variabl e immunodeficiency than patients without signs of liver disease. In most ca ses (21 of 26 patients) the liver disease was relatively mild, Three patien ts had granulomatous liver disease, with a relatively aggressive course in all three. Conclusion. Hepatobiliary disease is a frequent complication in primary hyp ogammaglobulinaemia. Liver disease in HCV RNA-negative patients usually has a mild course. HGV does not seem to be a major cause of chronic liver dise ase in these patients.