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目的探讨原发性胆汁性肝硬化(PBC)患者临床表现、自身抗体谱以及病理学特征。方法回顾性分析30例原发性胆汁性肝硬化患者临床资料,总结其发病特征、常见症状及体征、免疫学特征以及病理学特点。结果 30例患者最常见的症状为乏力(76.7%),最常见的体征为脾肿大(66.7%);抗线粒体抗体(AMA)及AMA-M2阳性率分别为90.0%和83.3%。丙氨酸转氨酶(ALT)(95.6±4.4)U/L、天门冬氨酸氨基转移酶(AST)(66.5±3.0)U/L、碱性磷酸酶(ALP)(286.5±8.5)U/L,总胆红素为(19.8±2.6)μmol/L。肝组织学检查以肉芽肿性胆管炎或非化脓性胆管炎为主。按照AMA及AMA-M2是否阳性进行分组,两组患者的临床症状、体征、实验室指标、病理分期及组织学特点比较,差异无统计学意义(P>0.05)。结论实验室检查结合相应的临床症状和体征可早期诊断原发性胆汁性肝硬化,对于抗线粒体抗体阴性的患者可行肝组织学检查明确诊断。
Objective To investigate the clinical manifestations, autoantibodies and pathological features of patients with primary biliary cirrhosis (PBC). Methods The clinical data of 30 patients with primary biliary cirrhosis were retrospectively analyzed. The incidence, common symptoms, signs, immunological characteristics and pathological features were summarized. Results The most common symptoms in 30 patients were fatigue (76.7%). The most common signs were splenomegaly (66.7%). The positive rates of AMA and AMA-M2 were 90.0% and 83.3% respectively. ALT (95.6 ± 4.4) U / L, AST (66.5 ± 3.0) U / L, ALP (286.5 ± 8.5) U / L , Total bilirubin (19.8 ± 2.6) μmol / L. Liver histology to granulomatous cholangitis or non suppurative cholangitis based. According to whether AMA and AMA-M2 were positive, the clinical symptoms, signs, laboratory indexes, pathological staging and histological features of two groups were not statistically different (P> 0.05). Conclusion Laboratory tests combined with the corresponding clinical symptoms and signs can be an early diagnosis of primary biliary cirrhosis, for anti-mitochondrial antibody-negative patients with liver biopsy can confirm the diagnosis.