原发性硬化性胆管炎27例临床病理特点分析

目的 总结原发性硬化性胆管炎(PSC)的临床病理特点,提高临床医师对该病的认识.方法 回顾性分析27例PSC患者的临床资料并随访患者当前情况.临床资料主要包括各项肝功能指标、自身抗体及免疫球蛋白,影像学及病理学检查结果将PSC患者分为大胆管型或小胆管型;治疗措施包括药物、内镜及手术治疗,治疗3～6个月后,ALT≤1.5倍、Tbil≤2倍、ALP≤2.5倍正常上限为有效.大胆管型PSC与小胆管型PSC的疗效比较采用非参数检验CMH法,P＜0.05为差异有统计学意义.结果 27例PSC患者中,男性7例,女性20例,平均年龄47.6岁.大胆管型PSC 18例,小胆管型PSC 9例.主要临床表现为黄疸(85.2%)、瘙痒(48.1%)、乏力(68.4%)、腹痛(40.7%)及发热(14.8%);常见体征包括:肝大(44.4%)、脾大(48.1%)、腹水(14.8%)等;胆红素升高10倍以上者占30.8%,IgG异常率81.8%、抗核抗体阳性率69.6%、抗中性粒细胞胞浆抗体阳性率52.9%;合并溃疡性结肠炎者占22.2%,干燥综合征患者占22.2%.经药物、内镜或手术治疗后,部分病例病情缓解,3例死亡;小胆管型PSC的疗效优于大胆管型PSC(66.7%比33.3%,x2=4.173,P=0.041).结论 本组少数PSC患者合并溃疡性结肠炎.小胆管型PSC治疗效果优于大胆管型PSC,提示早期诊治的必要性.

Clinical and pathological features of 27 cases of primary sclerosing cholangitis

Objective To elucidate clinical and pathological features of primary sclerosing cholangitis (PSC) in order to improve clinician's awareness of this rare disease. Methods We retrospectively analyzed clinical data and follow-up information of 27 PSC patients who were admitted to Beijing Friendship Hospital from January 1990 to November 2009. The patients were classified into classic PSC and small-duct PSC according to biochemistry and imaging results. After 3 to 6 months of therapy, those patients with serum ALT1 ≤ 1.5, TBil ≤ 2 and ALP ≤ 2.5 ULN were determined as good responders. The treatment results between the two groups were compared. Results 9 out of 27 cases of PSC were small duct PSC and 18 cases were large bile duct or classic PSC. Male patients (7) were less than females (20) and the average age was 47.6years. Main clinical symptoms included jaundice (85.2%), pruritis (48. 1%), fatigue (68.4 %), abdominal pain (40.7%) and fever(14.8%), main physical sign included hepatomegaly (44.4%), splenomegaly (48.1%) and ascites (14.8%). Laboratory features included elevated IgG (81.8%), positive ANA (69.6%) and pANCA (52.9%). 22% of these PSC patients had ulcerative colitis or Sjogren's syndrome. A small percentage of patients were responsive to standard therapy, of which small duct PSC had a better response than classic PSC (66.7 % vs 33.3%, P = 0.041). Conclusions Ulcerative colitis (22.2%) is not as common as reported by western countries. Small duct PSC has a better treatment response. Searching of effective treatment regimen for large bile duct PSC is warranted in future studies.