原发性硬化性胆管炎的诊断与治疗

Diagnosis and treatment of primary sclerosing cholangitis

目的探讨原发性硬化性胆管炎（PSC）的诊断方法和治疗方案。方法回顾性分析1991年1月至2009年8月北京协和医院收治31例PSC患者的临床资料，通过临床表现、辅助检查、手术方法等探讨该病临床病理特点和预后。结果28例患者表现为梗阻性黄疸，20例患者表现为发热，16例患者表现为腹痛；30例患者ALP升高；14例患者自身抗体阳性；15例患者血清肿瘤标志物异常，其中13例患者CA19—9升高（84～5000U／ml）。B超、CT、MRCP、ERCP检查确诊率分别为3／28、2／24、2／18、18／25。19例患者接受药物治疗，仅1例治疗后无明显不适；12例患者接受手术治疗，2例肝移植患者术后疗效较好；11例患者接受内镜治疗，1例患者治疗后无明显不适，其余患者症状有不同程度减轻。结论PSC临床表现多样，以CA19—9升高最常见。影像学检查中ERCP确诊率最高。常规的内、外科治疗只能缓解临床症状，肝移植的远期疗效较好。

Objective To investigate the diagnosis and treatment strategy of primary sclerosing cholangitis （PSC）. Methods From January 1991 to August 2009, 31 patients with PSC were treated at the Peking Union Medical College Hospital. A retrospective study was performed based on the clinical data of these patients to evaluate the methods of diagnosis, curative treatment and prognosis. Results Obstructive jaundice, fever and abdominal pain were the main symptoms of PSC, which accounted for 90% （28/31） , 65% （20/31） and 52% （ 16/31 ） , respectively. Thirty patients presented with an elevation of alkaline phosphatase. The positive rate of autoantibody was 45% （ 14/31 ）. The positive expression of serum tumor markers of 15 patients was elevated, and 13 of the 15 patients had an elevated expression of CA19-9 （84-5000 U/mL）. The diagnostic accuracies of B-mode ultrasound, computed tomography, magnetic resonance cholangiopancreatography, and endoscopic retrograde cholangiopancreatography （ERCP） were 3/28, 2/24, 2/18, 18/25, respectively. Nineteen patients received drug therapy and only one patient had no obvious discomfort after the treatment. Twelve patients received surgical treatment and the prognosis of two patients who underwent liver transplantation was good. Eleven patients received endoscopic treatment, only one patient had no obvious discomfort and the condition of the remaining 10 patients was alleviated after treatment. Conclusions PSC is usually characterized by an elevated expression of CA19-9. ERCP has a high diagnostic accuracy. Conventional treatments can only relieve the symptoms, whereas liver transplantation has a favorable long-term efficacy.