13例原发性硬化性胆管炎患者临床特征分析

摘要/Abstract

摘要： 目的探讨原发性硬化性胆管炎(PSC)患者的临床特点,提高临床医师的认识,减少误诊及漏诊率。方法回顾性分析2013年1月—2022年11月就诊于昆明医科大学第二附属医院确诊为PSC患者的临床特点、诊疗情况及预后。结果 13例患者中男性8例(66.7%),平均年龄46.1岁,病程3(1~26.5)月,其中9例(69.2%)诊断为大胆管型PSC。出现症状就诊者12例,无症状因肝功能不全就诊者1例,有症状者中腹痛9例(75.0%),瘙痒3例(25.0%),黄疸7例(58.3%),乏力5例(41.7%)。1例合并原发性胆汁性胆管炎,2例合并慢性胰腺炎,3例(23.1%)伴溃疡性结肠炎(UC)。13例患者均见肝生化指标异常,包括肝酶升高、高胆红素血症、低蛋白血症。免疫学检测结果显示,60%(6/10)PSC患者ANA阳性,pANCA 阳性者占25%。患者的MRCP表现主要是胆管粗细不均,管壁不均匀增厚,走行僵硬,部分狭窄、部分扩张,2例呈“枯树枝样”改变。10例行肝脏穿刺活检的病人仅1例表现为典型的“洋葱皮样”改变。13例患者予熊去氧胆酸(UDCA)治疗,其中2例联合激素治疗,经治疗后2例患者疗效不佳。本研究中位随访时间41(1~86)月,其中失访1例,1例死于急性肝衰竭。结论 PSC好发于中年男性,以大胆管型为主,临床表现无特异性,常伴发UC,可合并其他自身自身免疫性肝病,胆道造影是主要的诊断手段,UDCA可改善患者病情。

关键词: 原发性硬化性胆管炎, 诊断, 治疗, 预后

Abstract: Objective To investigate the clinical characteristics of patients with primary sclerosing cholangitis (PSC) to enhance understanding of the disease and reduce misdiagnosis and missed diagnoses. Methods A retrospective analysis was conducted on the clinical characteristics, diagnostic methods, treatment approaches, and prognosis of patients diagnosed with PSC at the Second Affiliated Hospital of Kunming Medical University from January 2013 to November 2022. Results Of the 13 patients, 8 (66.7%) were male, with a mean age of 46.1 years and a disease duration of 3 (1~26.5) months, Large-duct PSC was diagnosed in 9 patients (69.2%) . Twekve patients presented with symptoms, while one was asymptomatic due to hepatic insufficiency. Key symptoms included abdominal pain in 9 patients (75.0%), pruritus in 3 (25.0%), jaundice in 7 (58.3%), and fatigue in 5 (41.7%). One patients had concurrent primary biliary cholangitis, two had chronic pancreatitis, and three (23.1%) had ulcerative colitis. Liver biochemical abnormalities, including elevated liver enzymes, hyperbilirubinemia, and hypoproteinemia, were present in all patients. Immunological testing revealed ANA positivity in 60%(6/10) of patients and pANCA positivity in 25%. MRCP findings indicated bile duct wall irregularities, including uneven thickness, rigid courses, partial strictures and dilatations, and a “withered tree-like” appearance in 2 cases. Of the 10 patients who underwent liver biopsy, only one displayed the characteristic “Onion skin-like” fibrosis. All 13 patients were treated with ursodeoxycholic acid; 2 also received hormone therapy, and 2 showed limited response to treatment. The median follow-up duration was 41 months (1-86 months), with one patient lost to follow-up and one death due to acute liver failure. Conclusion PSC primarily affects middle-aged men and predominatly involves large-duct disease. Its clinical manifestations are nonspecific, frequently accompanied by ulcerative colitis, and may co-occur with other autoimmune liver diseases. Cholangiography remains the primary diagnostic tool, while treatment with ursodeoxycholic acid can improve clinical outcomes.

Key words: Primary sclerosing cholangitis, Diagnosis, Therapy, Prognosis

夏淑敏, 唐映梅, 徐加敏, 朱碧莲, 李秦念. 13例原发性硬化性胆管炎患者临床特征分析[J]. 肝脏, 2024, 29(11): 1396-1400.

XIA Shu-min, TANG Ying-mei, XU Jia-min, ZHU bi-lian, LI Qin-nian. Clinical characteristics of 13 patients with primary sclerosing cholangitis[J]. Chinese Hepatolgy, 2024, 29(11): 1396-1400.

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