原发性胆汁性肝硬化与弥漫结缔组织病的肝损伤

摘要

自身免疫性肝病是一组以肝脏为靶器官的自身免疫疾病,弥漫性结缔组织病的肝损伤是一组肝器官特异性的自身免疫病,越来越多的研究发现这两组疾病无论在临床表现以及基础免疫学的免疫异常变化都有相互联系.本文着重分析了弥漫性结缔与原发性胆汁性肝硬化的相关性,包括它们之间重叠存在的比例、临床症状、自身抗体的相关性,以及病理和免疫学的差异,目的 就是希望提高对原发性胆汁性肝硬化(PBC)及弥漫性结缔组织病(CTD)肝损伤的认识,进一步深入研究,提高诊断和治疗水平.%Autoimmune liver disease comprises of a group of liver-targeting autoimmune diseases, whereas diffuse connective tissue disease (DCTD) is a group of non-organ-specific autoimmune diseases. Increasing evidences have suggested that these two groups of disorders are closely related, not only by their clinical manifestations but also their basic immunological mechanisms. This paper focuses on correlation between DCTD and primary biliary cirrhosis (PBC), including their overlapping rate, clinical symptoms, autoantibody profile as well as their pathological and immunological differences, so as to update our knowledge of PBC and liver disease secondary to DCTD, and promote more profound investigation to improve our diagnosis and treatment of these disorders.