SUMMARY The frequency of HLA antigens was studied in 25 patients with primary sclerosing cholangitis and compared with a control group of 561 kidney donors. Fourteen patients also had ulcerative colitis. A significant increase in the frequency of HLA-B8 (60%) was found in the primary sclerosing cholangitis patients compared with controls (25%) (p<0-001). HLA-B8 was found in eight patients with ulcerative colitis. The frequency of HLA-B12 was significantly decreased (8%) compared with controls (30%) (p<0-02). Piecemeal necrosis was observed on liver histology in 66% of HLA-B8 positive and 50% of HLA-B8 negative patients. Low titres of serum autoantibodies were frequently found in the primary sclerosing cholangitis group but did not correspond to the presence of HLA-B8. Raised serum concentrations of IgM and IgG were not related to HLA-B8. This study has shown that in patients with primary sclerosing cholangitis there exists a disease susceptibility gene closely associated with the B locus of the major histocompatibility complex which may be modified by other factors such as ulcerative colitis. Patients with ulcerative colitis and HLA-B8 may be particularly liable to develop primary sclerosing cholangitis.Primary sclerosing cholangitis is an uncommon disease characterised by an intense inflammatory fibrosis usually involving the whole biliary system.'The aetiology of this disorder is unknown, although it is closely associated with ulcerative colitis which coexists in approximately two-thirds of patients with primary sclerosing cholangitis.'The associations between certain human leucocyte histocompatibility (HLA) antigens and an increased risk of certain diseases have recently been identified. HLA-B8 and HLA-DRW3, for example, have been closely associated with diseases involving immunological dysfunction such as autoimmune chronic active hepatitis.2 HLA-A3 has been closely associated with idiopathic haemochromatosis.3The purpose of the present study was to investigate possible associations between the HLA system and patients with primary sclerosing cholangitis, with and without coexisting ulcerative colitis. ' Address for correspondence: Dr R W Chapman, John Radcliffe Hospital, Headington, Oxford. Received for publication 13 April 1982 Methods PATIENTS Twenty-five consecutive patients (17 men and eight women) with primary sclerosing cholangitis who were admitted to the Royal Free Hospital for investigation were studied. The diagnosis was based on accepted criteria -namely, the demonstration of multiple strictures in the biliary system, the absence of previous biliary tract surgery, and the exclusion of bile duct cancer by at least two years' follow-up
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