自身免疫性肝病的组织病理学特点及其临床意义

自身免疫性肝病（AILD）可分为自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC）。肝活检对于AILD的诊断以及严重程度和预后判断至关重要。AIH-般以界面性肝炎（碎屑样坏死）、淋巴．浆细胞浸润和肝细胞玫瑰样花结为组织病理学特点．而PBC和PSC分别表现为胆管肉芽肿样破坏和胆管周围同心圆样纤维沉积（“洋葱皮样”）。除组织病理学证据外，AILD的诊断还需结合特征性临床、生化改变以及血清自身抗体和球蛋白水平综合判断。

Histopathologic Characteristics of Autoimmune Liver Disease and its Clinical Significance

Autoimmune liver disease （AILD） comprises autoimmune hepatitis （AIH）, primary biliary cirrhosis （PBC） and primary sclerosing cholangitis （PSC）. Liver biopsy is essential to establish the diagnosis and evaluate the disease severity and prognosis of AILD. AIH is generally characterized histopathologically by interface hepatitis （piecemeal necrosis）, infiltration of lymphoeytes and plasma cells, and rosette of hepatocytes, while the histopathologic characteristics of PBC and PSC are granulomatous destruction of the bile ducts and concentric periductal fibrosis （＂onion-skinning＂）, respectively. In the presence of compatible histopathologic findings, the diagnosis of AILD is based on its characteristic clinical and biochemical findings, circulating autoantibodies and abnormal level of serum globulins.