2025 年第 2 期 第 20 卷

原发性胆汁性胆管炎肝硬化合并系统性红斑狼疮致发热和反复血小板减少1例

A case of fever and recurrent thrombocytopenia attributed to cirrhosis of primary biliary cholangitis with systemic lupus erythematosus

作者：赵丹郭巧利任俊杰赵俊梅

英文作者：Zhao Dan Guo Qiaoli Ren Junjie Zhao Junmei

单位：山西医科大学第一医院消化内科，太原030001

英文单位：Department of Gastroenterology First Hospital of Shanxi Medical University Taiyuan 030001 China

关键词：原发性胆汁性胆管炎；系统性红斑狼疮；发热；血小板减少

英文关键词：Primarybiliarycholangitis;Systemiclupuserythematosus;Fever;Thrombocytopenia

• 摘要：

• 原发性胆汁性胆管炎会合并其他免疫系统疾病，当两种疾病同时出现时，容易引起复杂的并发症。本文报道了1例66岁女性原发性胆汁性胆管炎肝硬化期患者因出现不明发热、反复血小板减低入院，在除外感染、肿瘤、药物等多种病因后，最终诊断为原发性胆汁性胆管炎、系统性红斑狼疮、感染性心内膜炎的病例，并对其诊疗过程进行了分析。在抗感染等治疗的基础上给予马来酸阿伐曲泊帕升高血小板，继续激素同时联用环孢素及羟氯喹治疗系统性红斑狼疮后患者病情好转出院。半年后门诊随访复查心脏超声未见主动脉瓣膜赘生物，效果较好，供临床参考。

• Primary biliary cholangitis can combine other immune system diseases, when the two diseases appear at the same time, easily to cause complicated complications. This paper reports a case of a 66-year-old female patient with primary biliary cholangitis in the stage of liver cirrhosis who was admitted to the hospital due to unexplained fever and recurrent thrombocytopenia. After excluding infection, tumor, drug and other causes, the final diagnosis was primary biliary cholangitis, systemic lupus erythematosus, infective endocarditis, and the diagnosis and treatment process was analyzed. On the basis of anti-infection treatment, the patient was given avatrobopag maleate to increase platelet. Systemic lupus erythematosus was treated with corticosteroids, cyclosporine and hydroxychloroquine, and the patient′s condition improved and was discharged. Half a year later, no aortic valve vegetation was found by echocardiography in the outpatient follow-up, which was good for clinical reference.