Mitchison H C, Lucey M R, Kelly P J, Neuberger J M, Williams R, James O F
Department of Medicine, University of Newcastle Upon Tyne, England.
Gastroenterology. 1990 Sep;99(3):778-84. doi: 10.1016/0016-5085(90)90968-7.
The prognosis of patients with asymptomatic primary biliary cirrhosis has been uncertain. Cases of primary biliary cirrhosis in 95 patients from two centers are presented: 70 patients from a regional referral center (Freeman Hospital, FH) and 25 from an international tertiary referral center (King's College Hospital, KCH) with similar mean age at diagnosis and duration of follow-up (median, 12 months). During follow-up, 19 of 70 FH patients and 15 of 25 KCH patients developed symptoms (P less than 0.001); the mean time to symptom appearance was 43 months at FH and 35 months at KCH (P = 0.0012). Twenty-five of 95 patients died, 15 of 34 subjects who had developed symptoms (all liver-related deaths) and 10 of 61 who remained asymptomatic (all non-liver-related deaths) (P less than 0.001). Hepatic mortality was worse than among the normal population (P less than 0.05). A comparison of those patients developing symptoms with 111 at FH and 165 at KCH who presented with symptoms suggests no difference in survival once hepatic symptoms appear. In both centers, hepatic mortality was strongly related to symptom appearance in asymptomatic primary biliary cirrhosis: patients developing symptoms resemble those with symptoms at presentation. Other differences between the centers may reflect different referral patterns.
无症状原发性胆汁性肝硬化患者的预后一直不明确。本文报告了来自两个中心的95例原发性胆汁性肝硬化病例：70例来自地区转诊中心（弗里曼医院，FH），25例来自国际三级转诊中心（国王学院医院，KCH），诊断时平均年龄和随访时间相似（中位数为12个月）。随访期间，70例FH患者中有19例、25例KCH患者中有15例出现症状（P<0.001）；FH患者出现症状的平均时间为43个月，KCH患者为35个月（P=0.0012）。95例患者中有25例死亡，34例出现症状的患者中有15例（均为肝脏相关死亡），61例无症状患者中有10例（均为非肝脏相关死亡）（P<0.001）。肝脏死亡率高于正常人群（P<0.05）。将出现症状的患者与FH的111例和KCH的165例有症状的患者进行比较，结果显示一旦出现肝脏症状，生存率无差异。在两个中心，无症状原发性胆汁性肝硬化患者的肝脏死亡率都与症状出现密切相关：出现症状的患者与就诊时就有症状的患者相似。两个中心之间的其他差异可能反映了不同的转诊模式。
