淀粉样变性

在英国，年龄调整发病率为 5.1/100 万-12.8/100 万，每年约有 60 例新发病例。[4]Bergesio F, Ciciani AM, Santostefano M, et al. Renal involvement in systemic amyloidosis - an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant. 2007 Jun;22(6):1608-18.http://www.ncbi.nlm.nih.gov/pubmed/17395661?tool=bestpractice.com 在美国，原发性系统性淀粉样变性 (AL) 的患病率正在增加。美国的一项研究报告，AL 的患病率从 2007 年的 15.5/100 万，增加到了 2015 年的 40.5/100 万。[5]Quock TP, Yan T, Chang E, et al. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood Adv. 2018 May 22;2(10):1046-53.http://www.bloodadvances.org/content/2/10/1046.long?sso-checked=truehttp://www.ncbi.nlm.nih.gov/pubmed/29748430?tool=bestpractice.com 在同一项研究中，AL 的年发病率保持稳定（9.7-14.0/100 万人年）。[5]Quock TP, Yan T, Chang E, et al. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood Adv. 2018 May 22;2(10):1046-53.http://www.bloodadvances.org/content/2/10/1046.long?sso-checked=truehttp://www.ncbi.nlm.nih.gov/pubmed/29748430?tool=bestpractice.com 男性的 AL 患病率和发病率高于女性。[5]Quock TP, Yan T, Chang E, et al. Epidemiology of AL amyloidosis: a real-world study using US claims data. Blood Adv. 2018 May 22;2(10):1046-53.http://www.bloodadvances.org/content/2/10/1046.long?sso-checked=truehttp://www.ncbi.nlm.nih.gov/pubmed/29748430?tool=bestpractice.com 据报告，诊断时的平均年龄为 63 岁左右，但患者可能在任何年龄段就诊。尚无针对淀粉样变性的种族研究，但在黑人中，多发性骨髓瘤和意义未明单克隆免疫球蛋白血症 (MGUS) 的发病率是白人的两倍，根据推测，淀粉样变性的种族特点也是如此。[6]National Cancer Institute: Surveillance, Epidemiology, and End Results Program. Cancer stat facts: myeloma. 10 Apr 2018 [internet publication].https://seer.cancer.gov/statfacts/html/mulmy.html[7]Wadhera RK, Rajkumar SV. Prevalence of monoclonal gammopathy of undetermined significance: a systematic review. Mayo Clin Proc. 2010 Oct;85(10):933-42.http://www.ncbi.nlm.nih.gov/pubmed/20713974?tool=bestpractice.com[8]Landgren O, Weiss BM. Patterns of monoclonal gammopathy of undetermined significance and multiple myeloma in various ethnic/racial groups: support for genetic factors in pathogenesis. Leukemia. 2009 Oct;23(10):1691-7.https://www.nature.com/articles/leu2009134http://www.ncbi.nlm.nih.gov/pubmed/19587704?tool=bestpractice.com 所有单克隆丙种球蛋白病相关疾病的发病率在亚洲人群中较低。[4]Bergesio F, Ciciani AM, Santostefano M, et al. Renal involvement in systemic amyloidosis - an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant. 2007 Jun;22(6):1608-18.http://www.ncbi.nlm.nih.gov/pubmed/17395661?tool=bestpractice.com 过去几十年内，美国继发性淀粉样变性 (AA) 的发病率一直处于下降趋势，[9]Hazenberg BP, van Rijswijk MH. Where has secondary amyloid gone? Ann Rheum Dis. 2000 Aug;59(8):577-9.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1753216/pdf/v059p00577.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/10913049?tool=bestpractice.com 此类淀粉样变性仅占系统性淀粉样变性的 3%。在欧洲，AA 淀粉样变性可能占系统性淀粉样变性的 10%。