探讨原发性胆汁性肝硬化（PBC）患者的临床特征，为其诊治提供参考依据。

回顾性分析2005年10月至2016年11月西安交通大学第一附属医院收治的101例PBC患者临床资料。其中男7例，女94例；年龄17～80岁，中位年龄59岁。患者均签署知情同意书，符合医学伦理学规定。总结患者的年龄、性别、症状、体征等一般资料，分析入院后血常规、肝肾功能、凝血功能、自身抗体等诊断指标，以及治疗方式的选择。

PBC患者中女性占93.1%（94/101）。乏力、黄疸、呕血和黑便、皮肤瘙痒发生率分别占52.5%（53/101）、33.7%（34/101）、14.9%（15/101）、12.9%（13/101）。脾大、腹腔积液、肝病面容、蜘蛛痣体征分别占73.3%（74/101）、48.5%（49/101）、33.7%（34/101）、5.9%（6/101）。患者常出现三系细胞减少，血清ALT、AST、ALP、GGT、TB、DB异常和凝血功能障碍，其中DB异常发生率97.0%（98/101）。抗线粒体抗体（AMA）、抗核抗体（ANA）阳性率分别高达81.2%（82/101）、79.2%（80/101）。熊去氧胆酸等药物治疗占69.3%（70/101），肝移植占16.8%（17/101），其余对症治疗措施占13.9%（14/101）。

掌握PBC的临床特征，有助于其早期诊断及治疗。

To investigate the clinical characteristics of patients with primary biliary cirrhosis (PBC) and provide reference for the diagnosis and treatment of PBC.

Clinical data of 101 patients with PBC who were admitted to the First Affiliated Hospital of Xi'an Jiaotong University between October 2005 and November 2016 were retrospectively analyzed. Among them, 7 cases were males and 94 were females, aged 17-80 years old with a median age of 59 years old. The informed consents of all patients were obtained and the local ethical committee approval was received. General data such as age, gender, symptoms and physical signs were summarized. Diagnostic indicators after admission such as routine blood test, liver and renal function, coagulation function and autoantibody, and selection of therapy were analyzed.

The percentage of females among PBC patients was 93.1%(94/101). The incidence of asthenia, jaundice, hematemesis and melena, cutaneous pruritus was respectively 52.5%(53/101), 33.7%(34/101), 14.9%(15/101), 12.9%(13/101). The incidence of splenomegaly, peritoneal effusion, hepatic face and nevus araneus was respectively 73.3%(74/101), 48.5%(49/101), 33.7%(34/101) and 5.9%(6/101). Pancytopenia, abnormal levels of serum ALT, AST, ALP, GGT, TB and DB, and coagulation dysfunction were constantly observed. The incidence of DB abnormality was 97.0% (98/101). The positive rate of anti-mitochondrial antibody (AMA) and antinuclear antibody (ANA) was respectively up to 81.2%(82/101) and 79.2%(80/101). Among all the treatment, drug therapy such as ursodesoxycholic acid accounted for 69.3%(70/101), liver transplantation accounted for 16.8%(17/101) and other symptomatic therapies accounted for 13.9%(14/101).

Mastering the clinical characteristics of PBC patients contributes to early diagnosis and treatment of PBC.