抗磷脂综合征 (APS) 可在没有系统性自身免疫性疾病(原发性 APS)这一基础病或有其他自身免疫性疾病(同样称为继发性 APS)的患者中出现,尤其是在与系统性红斑狼疮 (SLE) 相关的情况下。APS 的真实发病率尚不清楚,且该病很可能诊断不足。据报告,APS 在正常健康人中的患病率为 1.0%~5.6%,且随着年龄增加而升高。[2]Shi W, Krilis SA, Chong BH, et al. Prevalence of lupus anticoagulant and anticardiolipin antibodies in a healthy population. Aust N Z J Med. 1990;20:231-236.http://www.ncbi.nlm.nih.gov/pubmed/2115326?tool=bestpractice.com[3]de Groot PG, Lutters B, Derksen RH, et al. Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. J Thromb Haemost. 2005;3:1993-1997.http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2005.01485.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/16102105?tool=bestpractice.com[4]Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun. 2000;15:145-151.http://www.ncbi.nlm.nih.gov/pubmed/10968901?tool=bestpractice.com与类风湿性关节炎相关的抗磷脂抗体患病率为 6%,而与系统性红斑狼疮 (SLE) 相关的为 30%~40%。[3]de Groot PG, Lutters B, Derksen RH, et al. Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. J Thromb Haemost. 2005;3:1993-1997.http://onlinelibrary.wiley.com/doi/10.1111/j.1538-7836.2005.01485.x/fullhttp://www.ncbi.nlm.nih.gov/pubmed/16102105?tool=bestpractice.com[5]Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: a systematic review. JAMA. 2006;295:1050-1057.http://jama.ama-assn.org/cgi/content/full/295/9/1050http://www.ncbi.nlm.nih.gov/pubmed/16507806?tool=bestpractice.com
多达 14% 的血栓形成患者报告出现抗磷脂抗体[5]Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: a systematic review. JAMA. 2006;295:1050-1057.http://jama.ama-assn.org/cgi/content/full/295/9/1050http://www.ncbi.nlm.nih.gov/pubmed/16507806?tool=bestpractice.com[6]Erkan D, Lockshin MD. What is antiphospholipid syndrome? Curr Rheumatol Rep. 2004;6:451-457.http://www.ncbi.nlm.nih.gov/pubmed/15527704?tool=bestpractice.com而有 20% 的复发性流产患者报告出现抗磷脂抗体。[7]Rai RS, Regan L, Clifford K, et al. Antiphospholipid antibodies and beta 2-glycoprotein-I in 500 women with recurrent miscarriage: results of a comprehensive screening approach. Hum Reprod. 1995;10:2001-2005.http://www.ncbi.nlm.nih.gov/pubmed/8567830?tool=bestpractice.com[8]Loizou S, Byron MA, Englert HJ, et al. Association of quantitative anticardiolipin antibody levels with fetal loss and time of loss in systemic lupus erythematosus. Q J Med. 1988;68:525-531.http://www.ncbi.nlm.nih.gov/pubmed/3252304?tool=bestpractice.com同时观察到女男比率范围为 5:1 至 2:1。[9]Jara LJ, Medina G, Vera-Lastra O, et al. The impact of gender on clinical manifestations of primary antiphospholipid syndrome. Lupus. 2005;14:607-612.http://www.ncbi.nlm.nih.gov/pubmed/16175932?tool=bestpractice.com大部分患者 (98%) 为白人,0.5% 为黑人,而 1.5% 属于其他人种。症状发作的平均年龄为约 34 岁,85% 的患者的确诊年龄为 15~50 岁。[9]Jara LJ, Medina G, Vera-Lastra O, et al. The impact of gender on clinical manifestations of primary antiphospholipid syndrome. Lupus. 2005;14:607-612.http://www.ncbi.nlm.nih.gov/pubmed/16175932?tool=bestpractice.com