原发性硬化性胆管炎28例临床特征及疗效回顾分析

Retrospective analysis of clinical characteristics and curative effect of 28 patients with primary sclerosing cholangitis

目的分析原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)的临床特征及诊治经验,以提高对该病的认识。方法对我院2000年1月-2010年12月收治确诊的28例PSC患者的临床特征、生化、免疫学、组织学及治疗等资料进行回顾性分析。结果 28例PSC患者中男21例(75%),女性7(25%),中位诊断年龄52岁(4～69岁);临床表现主要为黄疸(89.3%,25/28)、纳差(32.1%,9/28)、皮肤瘙痒(25.0%,7/28)、肝区疼痛(25.0%,7/28);实验室检查表现为总胆红素水平升高(89.3%,25/28);均以结合胆红素升高为主,肝酶升高(89.3%,25/28),包括谷丙转氨酶、谷草转氨酶、碱性磷酸酶及γ-谷氨酰转肽酶,但均以轻度升高为主;20例(90.9%,20/22)患者经胆管造影检查得到确诊,主要表现为肝内外胆管变细、粗细不均和/或走行僵直,其中经内镜逆行胰胆管造影(ERCP)确诊11例(100%,11/11)、经磁共振胰胆管造影(MRCP)确诊6例(85.7%,6/7)、经皮肝穿刺胆管造影(PTC)确诊2例(66.7%,2/3),经T管造影确诊1例(100%,1/1);其余8例(100%,8/8)患者经肝穿刺活检病理组织学检查确诊,主要表现为汇管区胆管周围炎症及纤维组织增生;25例患者经药物治疗后,13例(52%,13/25)生化指标有不同程度好转,3例经手术治疗的患者1例(33.3%,1/3)好转。结论 PSC发病机制未明,好发于男性,黄疸为主要症状,主要表现为肝酶和胆红素轻度升高,确诊应首选胆道造影检查,目前尚无特效治疗药物。

Objective To summarize the clinical characteristics and experience in the diagnosis and treatment of primary sclerosing cholangitis （PSC）. Methods Clinical data of 28 patients with PSC including clinical manifestations, PSC including clinical manifestations, laboratory tests, cholangiographies, pathological findings and the response to therapy were analyzed retrospectively from Jan. 2000 to Dec. 2010. Results 21 patients were males （75%） , and 7 pa- tients were females （25%）. The median age at diagnosis was 52 years old （4 -69 years old）. The most common symptoms were jaundice （89.3%, 25/28）, followed by anorexia （32.1%, 9/28）, pruritus （25.0%, 7/28）, hepatagia （25.0% , 7/28）. Liver enzyrne were elevated （89,3% , 25/28） , including alanine aminotransferase （ALT） , aspertate aminotransferase （AST）, alkaline phosphatase （ALP）, gamma glutamyl transpeptidase （GGT） and total bilirubin （TBIL; 89.3% , 25/28） , were obviously elevated in most patients. In all of the 28 patients, 20 were diagnosed by im- agings, including endoscopic retrograde cholangiopancreatography （ERCP） , magnetic resonance cholangiopancreatography （MRCP）, percutaneous transhepatic cholangiography （PTC） ,T-tube cholangiography, and the rest were diagnosed by liver biopsy. 13 patients out of 25 patients who received pharmacotherapy and 1 out of 3 who undergone surgery had biochemical improvements. Conclusion PSC is subjective to occur in men. Jaundice is the most common presentation and the biochemical tests such as ALT, AST, ALP, GGT and TBil are mildly elevated. Cholangiographies are the first chose to confirm the diagnosis of PSC, and there is no specific pharmaceutical for PSC.