Beswick D R, Klatskin G, Boyer J L
Gastroenterology. 1985 Aug;89(2):267-71.
Thirty-six patients presenting with asymptomatic primary biliary cirrhosis have been followed for a median period of 11.4 yr, extending by 5 yr a previously reported median follow-up study of 6 yr. Life table survival analysis indicates that the overall survival of this subgroup of patients with primary biliary cirrhosis continues to remain similar to that of the general population (p = 0.91). Over this period, 15 patients developed symptoms and 8 patients died, 6 from liver disease; 21 patients remained in an asymptomatic state. Portal granulomas on initial liver biopsy were the only finding that correlated with a normal survival and a continued asymptomatic state (p = 0.03). In contrast, associated autoimmune disorders (thyroiditis, sicca syndrome, CRST syndrome, Raynaud's phenomenon) correlated with decreased survival (p = 0.01). No other clinical, laboratory, or histologic features correlated with survival or the development of symptoms. This extended follow-up study (median 11.4 yr) indicates that many patients with asymptomatic primary biliary cirrhosis have a benign outcome. Although 42% developed signs or symptoms of progressive disease at variable times up to 14 yr from presentation, the group survival remained similar to the general population.
36例无症状原发性胆汁性肝硬化患者的中位随访时间为11.4年，将之前报道的6年中位随访研究延长了5年。生命表生存分析表明，该原发性胆汁性肝硬化亚组患者的总体生存率仍与普通人群相似（p = 0.91）。在此期间，15例患者出现症状，8例患者死亡，其中6例死于肝脏疾病；21例患者仍无症状。初次肝活检时的门脉肉芽肿是唯一与正常生存及持续无症状状态相关的发现（p = 0.03）。相反，相关自身免疫性疾病（甲状腺炎、干燥综合征、CRST综合征、雷诺现象）与生存率降低相关（p = 0.01）。没有其他临床、实验室或组织学特征与生存或症状的出现相关。这项延长的随访研究（中位11.4年）表明，许多无症状原发性胆汁性肝硬化患者预后良好。尽管42%的患者在出现症状后的14年内不同时间出现了进行性疾病的体征或症状，但该组患者的生存率仍与普通人群相似。
