CAJ | 학술논문

目的：比较研究PBC/AIH重叠综合征和单纯PBC或AIH患者的临床表现、生化检查、免疫学检查及病理学特点。方法：选取自身免疫性肝病患者86例，对其一般资料、临床表现、实验室检查、病理学改变及治疗转归进行回顾性分析。结果：86例自身免疫性肝病患者中有10例AIH/PBC诊断为重叠综合征；生化检查其血清碱性磷酸酶、γ-谷胺酰氨基转移酶、总胆红素、直接胆红素、谷丙转氨酶及谷草转氨酶呈明显升高；特异性免疫指标ANA阳性6例，AMA阳性8例，AMA-M2阳性8例，SMA阳性2例；病理学特征表现有80%的患者可见界面炎/碎屑坏死，有患者出现桥接样坏死，纤维组织增生多见，70%的患者有浆细胞浸润，患者均有不同程度的胆管改变。结论：PBC/AIH重叠综合征不仅具备AIH的特点，同时还具备PBC的特点。
목적：비교연구PBC/AIH중첩종합정화단순PBC혹AIH환자적림상표현、생화검사、면역학검사급병이학특점。방법：선취자신면역성간병환자86례，대기일반자료、림상표현、실험실검사、병이학개변급치료전귀진행회고성분석。결과：86례자신면역성간병환자중유10례AIH/PBC진단위중첩종합정；생화검사기혈청감성린산매、γ-곡알선안기전이매、총담홍소、직접담홍소、곡병전안매급곡초전안매정명현승고；특이성면역지표ANA양성6례，AMA양성8례，AMA-M2양성8례，SMA양성2례；병이학특정표현유80%적환자가견계면염/쇄설배사，유환자출현교접양배사，섬유조직증생다견，70%적환자유장세포침윤，환자균유불동정도적담관개변。결론：PBC/AIH중첩종합정불부구비AIH적특점，동시환구비PBC적특점。
Objective:To study the features of clinic,biochemistry,immunology and pathology in PBC/AIH and PBC(or AIH). Methods:Eighty-six patients with autoimmunic liver disease were studied to compare PBC, AIH and PBC/AIH overlap syndome in the clinical symptoms, signs, labarotary findings and histological features. Results: Ten cases were diagnosed as AIH\/PBC overlap syndrome in eighty-six patients with autoimmune liver disease.The biochemical tests results were the following indexes increased significantly in serum alkaline phosphatase, gamma glutamyl aminotransferase, total bilirubin, direct bilirubin, alanine aminotransferase as aspartate aminotransferase; The specific immunological marker were:ANA positive in six cases, AMA positive six cases, AMA-M2 positive in four cases, SMA positive two cases; The pathological features were found as: visible interface inflammation in 80% cases, clastic necrosis and bridging necrosis, fibrous tissue proliferation in some cases, plasma cell infiltration in 70% cases. In addition, all patients were observed the lesion of bile duct in different degree. Conclusion: PBC/AIH overlap syndrome has not only the characteristics of AIH, but also the characteristics of PBC.