在英国有8 500例,在美国有30 000例,而全世界有70 000例。[5]Cystic Fibrosis Trust. https://www.cysticfibrosis.org.uk/ (last accessed 31 August 2016).https://www.cysticfibrosis.org.uk/[6]The Cystic Fibrosis Foundation. http://www.cff.org/ (last accessed 31 August 2016).http://www.cff.org/在白种人中的发病率为1/3 000;而在非洲、西班牙、亚洲裔人群中发病率较低。在欧洲裔人群中最为常见。[7]Eigen H, Rosenstein BJ, FitzSimmons S, et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr. 1995;126:515-523.http://www.ncbi.nlm.nih.gov/pubmed/7699528?tool=bestpractice.com研究提示,新生儿筛查制度实施后该病的发病率可能会有所下降。[8]Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. 2008;358:973-974.http://www.ncbi.nlm.nih.gov/pubmed/18305279?tool=bestpractice.com肺部疾病是该病发病和死亡的最常见原因。