原发性胆汁性肝硬化临床分析(附49例报告)

Clinical characteristics of primary biliary cirrhosis: Analysis of 49 cases

目的分析原发性胆汁性肝硬化(primarybiliarycirrhosis,PBC)的临床特点、常伴疾病及治疗反应,提高对PBC的认识和治疗水平。方法对因PBC住院的49例患者临床资料进行回顾性分析。结果①49例患者中女性33例,男女比例为1:2.06;确诊时的平均年龄为(45.8±15)岁,发病到确诊的时间平均为4.8年(1个月至18年)。②症状和体征:黄疸81.6%、瘙痒75.5%、乏力67.3%、脾大73.5%、肝大61.2%、腹水44.9%。③实验室检查:全部病例的血清碱性磷酸酶(ALP)和r-谷氨酰转肽酶(r-GT)都升高,43例血清胆红素(TBil)显著升高,42例谷草转氨酶(AST)升高,39例谷丙转氨酶(ALT)升高。ALT和AST均为轻中度升高,且以AST升高明显。37例患者同时存在高脂血症。38例患者(占77.6%)血清IgM升高、37例患者检测了抗线粒体抗体(AMA)及其M2亚型,AMA阳性34例(占91.9%),M2亚型阳性33例(占89.2%)。④合并干燥综合征(SS):符合干燥综合征国际分类标准的有15例(占30.6%)。⑤肝穿刺病理取材满意5例,病理组织学分期III期3例,II期2例。⑥治疗反应:常规保肝治疗效果不佳,40例加用熊去氧胆酸者能明显改善胆汁淤积指标,皮质激素、免疫抑制剂治疗效果不肯定。结论PBC多见于中年女性,黄疸、瘙痒、肝脾肿大,血清ALP、r-GT和IgM水平升高,AMA和(或)M2亚型阳性有助于诊断本病,肝活检病理学检查对诊断和组织学分期有帮助,熊去氧胆酸可取得一定的治疗效果。重视对本病的认识是提高早期诊断和提高治疗效果的关键。

Objective In order to facilitate our recognition of primary biliary cirrhosis （PBC）, we studied PBC＇s clinical features, main concomitant diseases and the responses to therapy. Methods clinical data of 49 patients with PBC were analyzed retrospectively. Results （1）Among 49 patients 33 were female, with a male/female ratio of 1：2.06; The mean age was 45.8 ± 15.0 years at the time when definite diagnosis was made. The mean time interval between the first symptom arise to the time of diagnosis was about 4.8 years. （2）The major symptoms in these patients were jaundice 81.6% （40/49） ,pruritus 75.5% （ 37/49 ） ,fatigue 67.3% （ 33/49 ）. The major signs are splenomegaly 73.5% （ 36/49 ） ,hepatomegaly 61.2% （ 30/49 ） , ascites 44.9% （ 22/49 ）. （3）I.aboratory abnormality： hyperbilirubinemia 87.8% （ 43/49 ） ; Predominant elevations of r-glutamyltranspeptidase （ r-GT ） 100% （49/49） , alkaline phosphatase （ ALP ） 100% （ 49/49 ）. ALT 79.6% （ 39/49 ） , AST 83.7% （ 42/49 ）, IgM 77.6% （ 38/49 ） ; Antimitochondrial antibody （ AMA ） of 34 cases in 37 patients were positive （91.9%） and Ms subtype was positive in 33 patients （89.2%）. （4）15 of 49 cases accompanied with sjOgren syndrome （ 30.6% ） （5）The pathologic diagnosis of liver biopsy in 5 of 49 cases was hepatic cell necrosis in 3 cases and fibrosis in 2 cases . （6）The responses to therapy： ursodeoxycholic acid （ UDSA ） was effective in 40 patients（81.6%）. Conclusions PBC mainly affects middle aged women. Jaundice, pruritus, splenomegaly and hepato- megaly are their clinical features. The elevated level of ALP, r -GT, IgM , AMA and/or Ms subtype positive are typical in diagnosis of PBC. Liver biopsy can help to make a identify diagnosis and carry on pathological staging. UDSA can improve curative effects of PBC. It＇s important to pay much more attention to the recognition of PBC for early diagnosis and good effects of treatment.