原发性硬化性胆管炎的内镜治疗及预后分析

Endoscopic treatment and prognosis of primary sclerosing cholangitis

摘要:

目的 探讨原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）患者行经内镜逆行胰胆管造影术（endoscopic retrograde cholangiopancreatography，ERCP）的诊疗效果及预后相关因素。方法 纳入2009年5月—2020年5月西京医院接诊的PSC病例。回顾性收集人口学、临床症状、实验室和影像学检查、ERCP诊疗等数据，探讨ERCP治疗人群特点及临床疗效，随访观察疾病进展、无移植生存期和总体生存期等。结果 共纳入74例PSC患者，中位年龄53岁，男性占54.1%（40/74），合并胆管显性狭窄、炎症性肠病和其他自身免疫性肝病患者分别占32.4%（24/74）、18.9%（14/74）和17.6%（13/74），接受ERCP患者占36.5%（27/74）。Logistic回归分析显示，高总胆红素（OR=12.33，95%CI：1.24~122.63，P=0.032）和合并胆管显性狭窄（OR=24.67，95%CI：3.40~178.88，P=0.002）是ERCP诊疗的独立危险因素。ERCP操作和临床成功率均为96.3%（26/27）。截至最后一次随访，患者进展为肝硬化、胆管癌、行肝移植及死亡比例分别为9.5%（7/74）、4.1%（3/74）、5.4%（4/74）和18.9%（14/74）。随访患者（n=54）的五年生存率为83.3%。接受ERCP患者与未接受ERCP患者相比，无移植生存期（P=0.933）和总体生存期（P=0.608）差异均无统计学意义。瘙痒患者 （HR=5.30，95%CI：1.50~18.90，P=0.010）无移植生存期更短。结论 PSC患者合并炎症性肠病较少而合并自身免疫性肝病的比例较高。高总胆红素或胆管显性狭窄患者接受ERCP治疗占比更高，其操作及临床成功率满意，但ERCP对长期预后无显著影响。瘙痒患者的无移植生存期较短。

Abstract:

Objective To investigate the outcome and prognostic factors associated with endoscopic retrograde cholangiopancreatography (ERCP) in patients with primary sclerosing cholangitis (PSC). Methods PSC patients admitted to Xijing Hospital from May 2009 to May 2020 were included. Data of demographics, clinical symptoms, laboratory and imaging tests, and ERCP consultations were collected to explore the population characteristics and clinical efficacy of ERCP treatment, and to follow up disease progression, transplant-free survival, and overall survival. Results A total of 74 patients with PSC were included in this study, with a median age of 53 years, 54.1% (40/74) male. Patients combined with bile duct dominant stenosis, inflammatory bowel disease (IBD), and another autoimmune liver disease were 32.4% (24/74), 18.9% (14/74), and 17.6% (13/74), respectively, and those undergoing ERCP were 36.5% (27/74). Logistic regression analysis showed that high total bilirubin (OR=12.33, 95%CI: 1.24-122.63, P=0.032) and bile duct dominant stenosis (OR=24.67, 95%CI: 3.40-178.88, P=0.002) were independent high-risk factors for ERCP consultation. The operation and clinical success rates of ERCP were both 96.3% (26/27). As of the last follow-up, the proportions of patients progressing to cirrhosis, bile duct cancer, liver transplantation and death were 9.5% (7/74), 4.1% (3/74), 5.4% (4/74) and 18.9% (14/74), respectively. The five-year survival rate of the follow‑up patients (n=54) was 83.3%. The differences in transplant-free survival (P=0.933) and overall survival (P=0.608) between ERCP patients and non-ERCP patients were not statistically significant. Transplant-free survival of those who were companied with pruritus (HR=5.30, 95%CI: 1.50-18.90, P=0.010) was shorter. Conclusion PSC patients have higher proportion of IBD and less autoimmune liver disease. Higher proportion of patients with higher total bilirubin or bile duct dominant stenosis receive ERCP. While the short-term efficacy of ERCP is satisfactory, the long-term prognosis is still suboptimal. Patients with pruritus have a shorter transplant-free survival.