原发性硬化性胆管炎12例临床分析

Clinical analysis of 12 cases of primary sclerosing cholangitis

目的总结原发性硬化性胆管炎(PSC)的临床特征,提高对该病的认识水平。方法回顾分析北京友谊医院2005年11月～2009年6月诊断为PSC的12例患者的临床表现、实验室检查、影像、病理组织学特点、治疗情况。结果 12例患者中女性8例,男性4例,平均发病年龄43.8岁。以黄疸、乏力、皮肤瘙痒、纳差、肝脾大为主要表现。ALP平均升高3倍,GGT平均升高9倍,66.7%免疫球蛋白IgG升高,抗核抗体(ANA)阳性7例(58.3%),抗中性粒细胞胞浆抗体(ANCA)阳性5例(41.7%)。8例肝穿检查者中7例可见胆管周围纤维化、基底膜增厚典型PSC改变。熊去氧胆酸(UDCA)治疗后,9例生化指标改善,3例合并肝硬化的患者中2例无效,1例合并胆管癌的患者无效。结论对不明原因的梗阻性黄疸、ALP和GGT升高的病例,及早给予磁共振胰胆管造影(MRCP)或进一步行内镜逆行胰胆管造影(ERCP)、肝穿病理检查,有助于PSC的早期诊断。

Objective Clinical features of 12 cases of primary sclerosing cholangitis（PSC）,a rare disease in China,were analyzed in order to improve the recognition of the disease here.Methods 12 cases of PSC identified by radiological and/ or pathological examinations from November 2005 to June 2009 were analysed retrospectively.Results Of 12 patients,8 cases were female and 4 cases were male.The mean age of onset was 43.8 years old.The majority of PSC patients presented with jaundice,fatigue,pruritus,anorexia,and splenomegaly.Elevation of alkaline phosphatase（ALP） and r-glutamyl transpeptidase（GGT） occured in 100% of cases,and a mild to moderate elevation of aminotransferase levels was also pres-ented.Bilirubin levels fluctuated.Antinuclear antibody（ANA） was positive in 7 cases.Antineutrophil cytoplasmic antibody（ANCA） was positive in 5 cases.The immuno-globulin G level was high in 8 cases.7 of 8 cases who had a liver biopsy had the typical findings of PSC.After therapy with ursodeoxycholic acid（UDCA）,9 cases had improvements in biochemi-cal test,2 cases of cirrhosis and 1 case of cholangiocarcinoma had no effect.Conclusion The patients with unknown causes cholestasis and elevated ALP and GGT should exclude PSC,magnetic resonance cholangiopancreaticography（MRCP）,endo-scopic retrograde cholangiopancreaticography（ERCP） or liver biopsy examination should be carried out.