Sjogren 综合征是一种系统性自身免疫性疾病,可能是系统性自身免疫性风湿病中最常见的。男女患病率之比为 1:9,人群总患病率为 0.5%-1.56%,具体取决于所使用的诊断标准。[8]Manthorpe R, Manthorpe T. Primary Sjogren's syndrome - epidemiology and prognosis. Akt Rheumatol. 2005;30:27-31.[9]Fox RI. Sjogren's syndrome. Lancet. 2005;366:321-331.http://www.ncbi.nlm.nih.gov/pubmed/16039337?tool=bestpractice.com[10]Kabasakal Y, Kitapciglu G, Turk T, et al. The prevalence of Sjogren's syndrome in adult women. Scand J Rheumatol. 2006;35:379-383.http://www.ncbi.nlm.nih.gov/pubmed/17062438?tool=bestpractice.com 据估计,美国有 2-4 百万人患有此病。[2]Fox PC. Autoimmune diseases and Sjogren's syndrome, an autoimmune exocrinopathy. Ann N Y Acad Sci. 2007;1098:15-21.http://www.ncbi.nlm.nih.gov/pubmed/17332090?tool=bestpractice.com约 60% 患者的疾病继发于类风湿关节炎、系统性红斑狼疮 (systemic lupus erythematosus, SLE) 或系统性硬化症。[11]Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjogren syndrome. Arch Intern Med. 2004;164:1275-1284.http://jamanetwork.com/journals/jamainternalmedicine/fullarticle/217138http://www.ncbi.nlm.nih.gov/pubmed/15226160?tool=bestpractice.com已确定两个高峰发病年龄段,分别是初潮后(20-40 岁)和绝经后(50 多岁)。[9]Fox RI. Sjogren's syndrome. Lancet. 2005;366:321-331.http://www.ncbi.nlm.nih.gov/pubmed/16039337?tool=bestpractice.com然而,一项挪威的研究报告表明,71-74 岁年龄组患者 Sjogren 综合征的发病率是 40-44 岁年龄组患者的 7 倍。[12]Haugen AJ, Peen E, Hulten B, et al. Estimation of the prevalence of primary Sjogren's syndrome in two age-different community-based populations using two sets of classification criteria: the Hordland Health Study. Scand J Rheumatol. 2008;37:30-34.http://www.ncbi.nlm.nih.gov/pubmed/18189192?tool=bestpractice.com一项美国的研究发现,一组年龄在 65-84 岁的 2481 名个体中,27%述有眼干或口干。[13]Schein OD, Hochberg MC, Muñoz B, et al. Dry eye and dry mouth in the elderly: a population-based assessment. Arch Intern Med. 1999;159:1359-1363.http://jamanetwork.com/journals/jamainternalmedicine/fullarticle/485064http://www.ncbi.nlm.nih.gov/pubmed/10386512?tool=bestpractice.com两个研究组报告了同卵双生子共患 Sjogren 综合征,但尚不明确在双胞胎患者中该病是否存在一致性。[14]Scofield RH, Kurien BT, Reichlin M. Immunologically restricted and inhibitory anti-Ro in monozygotic twins. Lupus. 1997;6:395-398.http://www.ncbi.nlm.nih.gov/pubmed/9175026?tool=bestpractice.com[15]Bolstad AI, Haga HJ, Wassmuth R, et al. Monozygotic twins with primary Sjogren's syndrome. J Rheumatol. 2000;27:2264-2266.http://www.ncbi.nlm.nih.gov/pubmed/10990245?tool=bestpractice.com