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目的:分析原发性胆汁性肝硬化(PBC)患者的临床表现,试验室检查及治疗情况。方法:回顾性分析48例PBC患者临床资料。结果:93.8%是中年女性患者,平均年龄53.2±8.73。主要的临床表现包括肝功能异常(ALT、GGT、AKP升高)95.8%,乏力纳差83.3%,黄疸79.2%,瘙痒66.6%,肝肿大62.5%,脾大58.3%等。常合并干燥综合征(25%),类风湿关节炎(16.7%)等自身免疫性疾病及结缔组织疾病。所以患者AMA及AMA-M2均为阳性。全部病例使用熊去氧胆酸治疗,但仅31.3%患者病情有不同程度好转。结论:加强对PBC的认识,重视对AMA或AMA-M2的检测,尤其对长期不明原因肝功能异常的女性患者。
Objective: To analyze the clinical manifestations, laboratory tests and treatment of patients with primary biliary cirrhosis (PBC). Methods: A retrospective analysis of 48 cases of PBC clinical data. Results: 93.8% were middle-aged female patients with an average age of 53.2 ± 8.73. The main clinical manifestations include liver dysfunction (ALT, GGT, AKP elevated 95.8%), anorexia 83.3%, jaundice 79.2%, pruritus 66.6%, hepatomegaly 62.5%, splenomegaly 58.3%. Often combined with Sjogren’s syndrome (25%), rheumatoid arthritis (16.7%) and other autoimmune diseases and connective tissue diseases. So patients AMA and AMA-M2 are positive. Ursodeoxycholic acid was used in all cases, but only 31.3% of the patients had varying degrees of improvement. Conclusion: To strengthen the understanding of PBC, pay attention to the detection of AMA or AMA-M2, especially for long-term unexplained liver dysfunction in female patients.