抗肾小球基底膜肾病相当罕见,估计发病率为每百万人1至2例。[4]Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999;10:2446-2453.http://jasn.asnjournals.org/cgi/content/full/10/11/2446http://www.ncbi.nlm.nih.gov/pubmed/10541306?tool=bestpractice.com占肾小球肾炎所有病因的1%-2%。[3]Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. 2002;95:1411-1418.http://www.ncbi.nlm.nih.gov/pubmed/12597309?tool=bestpractice.com在一个研究队列中,表现为肺肾综合征的患者抗GBM抗体阳性率为15%。[5]Niles JL, Bottinger SP, Saurina GT, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996;156:440-445.http://www.ncbi.nlm.nih.gov/pubmed/8607730?tool=bestpractice.com30%至50%的抗GBM肾病患者同时有抗中性粒细胞抗体阳性。[1]Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003;64:1535-1550.http://www.ncbi.nlm.nih.gov/pubmed/12969182?tool=bestpractice.com[5]Niles JL, Bottinger SP, Saurina GT, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996;156:440-445.http://www.ncbi.nlm.nih.gov/pubmed/8607730?tool=bestpractice.com该病在男性多发,男性患者约占60%,女性患者约占40%。[3]Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. 2002;95:1411-1418.http://www.ncbi.nlm.nih.gov/pubmed/12597309?tool=bestpractice.com有两个发病年龄高峰:20 至 30 岁和 60 至 70 岁。[5]Niles JL, Bottinger SP, Saurina GT, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996;156:440-445.http://www.ncbi.nlm.nih.gov/pubmed/8607730?tool=bestpractice.com约83%的病例发生于白种人。[3]Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. 2002;95:1411-1418.http://www.ncbi.nlm.nih.gov/pubmed/12597309?tool=bestpractice.com在中国,该病发病率是每年每百万人口0.6例。[6]Li FK, Tse KC, Lam MF, et al. Incidence and outcome of antiglomerular basement membrane disease in Chinese. Nephrology. 2004;9:100-104.http://www.ncbi.nlm.nih.gov/pubmed/15056270?tool=bestpractice.com