总人群中PBC总的患病率大约为35/10万，每年的发病率大约为5/10万。[2]Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000;119:1631-1636.http://www.ncbi.nlm.nih.gov/pubmed/11113084?tool=bestpractice.com它在女性中的患病率远远大于在男性中的患病率（可高达10倍的差别）[2]Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000;119:1631-1636.http://www.ncbi.nlm.nih.gov/pubmed/11113084?tool=bestpractice.com[3]James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999;30:390-394.http://onlinelibrary.wiley.com/doi/10.1002/hep.510300213/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/10421645?tool=bestpractice.com[4]Gershwin ME, Selmi C, Worman HJ, et al. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology. 2005;42:1194-1202.http://onlinelibrary.wiley.com/doi/10.1002/hep.20907/fullhttp://www.ncbi.nlm.nih.gov/pubmed/16250040?tool=bestpractice.com但男性患者和女性患者的临床表现并没有不同。[5]Lucey MR, Neuberger JM, Williams R. Primary biliary cirrhosis in men. Gut. 1986;27:1373-1376.http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=3792920http://www.ncbi.nlm.nih.gov/pubmed/3792920?tool=bestpractice.comPBC 诊断的峰值年龄介于 55 和 65 岁，不过在 20 几岁之后就可患该病，并且更年轻的患者可能发生更具侵袭性、对治疗反应更差的疾病。有过本病在儿童中发病的个案报道。关于PBC种族差异的研究数据有限，但相同地理环境下的不同民族群体，其发病风险大致相似（虽然不完全相同）（此发现源于一项将环境因素纳入疾病发病机制的研究）。这种疾病可能发生在所有族群中，PBC在欧洲国家的发病率和患病率与美国相似，[3]James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999;30:390-394.http://onlinelibrary.wiley.com/doi/10.1002/hep.510300213/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/10421645?tool=bestpractice.com这种疾病在非洲和亚洲国家/地区较为少见（虽然没有充分的研究）。欧洲和美国PBC的发病率和患病率仍不断在增加。[2]Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000;119:1631-1636.http://www.ncbi.nlm.nih.gov/pubmed/11113084?tool=bestpractice.com[3]James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999;30:390-394.http://onlinelibrary.wiley.com/doi/10.1002/hep.510300213/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/10421645?tool=bestpractice.com目前尚不清楚这一增加是否真实，抑或其为临床医生对该疾病认识程度增加和血清学诊断工具容易获得的一个结果。PBC发病风险最高的人群是欧洲和美国超过50岁的女性居民，在该组人群中的患病率可高达1/750。[3]James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology. 1999;30:390-394.http://onlinelibrary.wiley.com/doi/10.1002/hep.510300213/pdfhttp://www.ncbi.nlm.nih.gov/pubmed/10421645?tool=bestpractice.com
