Suppressor T-cell deficiency in primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis is considered to be an autoimmune disease of the liver in which there is an association with the HLA phenotypes B8 and DR3 and in which circulating autoantibodies occur. Abnormalities of immune regulation may be present but whether or not they are primary or acquired is not known. This report is of a patient with primary sclerosing cholangitis who was homozygous for HLA B8 DR3, had a circulating antinuclear antibody, and a defect in nonspecific suppressor T-cell activity despite glucocorticosteroid treatment. Nevertheless, family studies revealed no evidence of an immunoregulatory defect in first-degree relatives despite the presence of Raynaud's phenomenon and malignancy in two sisters.

Autoantibody Profile of Primary Sclerosing Cholangitis

“…Spontaneous autoantibody re sponses are T-cell-dependent [27], and the suppressor T cell deficiency described in PSC patients [28] could have a role in the induction of the multiple autoantibody produc tion presented herein. Alternatively, the various autoanti bodies in PSC patients could be induced by tissue damage and liberation of autoantigens.…”