Kilby A E, Krawitt E L, Albertini R J, Chastenay B F, John A
Department of Medicine, University of Vermont, Burlington 05405.
Dig Dis Sci. 1991 Sep;36(9):1213-6. doi: 10.1007/BF01307511.
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.
原发性硬化性胆管炎被认为是一种肝脏自身免疫性疾病，它与HLA表型B8和DR3相关，且会出现循环自身抗体。可能存在免疫调节异常，但尚不清楚这些异常是原发性的还是后天获得性的。本报告介绍了一名原发性硬化性胆管炎患者，该患者为HLA B8 DR3纯合子，有循环抗核抗体，尽管接受了糖皮质激素治疗，但非特异性抑制性T细胞活性仍存在缺陷。然而，家族研究显示，尽管患者的两个姐妹患有雷诺现象和恶性肿瘤，但一级亲属中没有免疫调节缺陷的证据。
