在美国和欧洲,乳糜泻是常见疾病。根据一些设计精良的研究,许多国家/地区已发现相对一致的患病率,汇总的全球血清阳性率和活检证实的患病率分别为 1.4% 和 0.7%。[1]Singh P, Arora A, Strand TA, et al. Global prevalence of celiac disease: systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2018 Jun;16(6):823-36.http://www.ncbi.nlm.nih.gov/pubmed/29551598?tool=bestpractice.com[2]Sood A, Midha V, Sood N, et al. Prevalence of celiac disease among school children in Punjab, North India. J Gastroenterol Hepatol. 2006 Oct;21(10):1622-5.http://www.ncbi.nlm.nih.gov/pubmed/16928227?tool=bestpractice.com[3]Ertekin V, Selimoglu MA, Kardas F, et al. Prevalence of celiac disease in Turkish children. J Clin Gastroenterol. 2005 Sep;39(8):689-91.http://www.ncbi.nlm.nih.gov/pubmed/16082278?tool=bestpractice.com 然而,尽管血清阳性率大致相同,但在南美洲、中东、土耳其和撒哈拉以南的非洲地区,经活检证实的乳糜泻较不常见。[1]Singh P, Arora A, Strand TA, et al. Global prevalence of celiac disease: systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2018 Jun;16(6):823-36.http://www.ncbi.nlm.nih.gov/pubmed/29551598?tool=bestpractice.com 除马来西亚和越南外,还缺乏远东地区(包括中国、日本和东南亚)的人群研究。[4]Yap TW, Chan WK, Leow AH, et al. Prevalence of serum celiac antibodies in a multiracial Asian population: a first study in the young Asian adult population of Malaysia. PLoS One. 2015 Mar 23;10(3):e0121908.https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0121908http://www.ncbi.nlm.nih.gov/pubmed/25799401?tool=bestpractice.com[5]Cummins AG, Roberts-Thomson IC. Prevalence of celiac disease in the Asia-Pacific region. J Gastroenterol Hepatol. 2009 Aug;24(8):1347-51.http://www.ncbi.nlm.nih.gov/pubmed/19702902?tool=bestpractice.com北美的患病率经数十年的上升,近年来乳糜泻的患病率似乎趋于稳定。[6]Choung RS, Unalp-Arida A, Ruhl CE, et al. Less hidden celiac disease but increased gluten avoidance without a diagnosis in the United States: findings from the National Health and Nutrition Examination Surveys from 2009 to 2014. Mayo Clin Proc. 2016 Dec 5 [Epub ahead of print].https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5459670/http://www.ncbi.nlm.nih.gov/pubmed/28017411?tool=bestpractice.com
女性略微更易患乳糜泻。[1]Singh P, Arora A, Strand TA, et al. Global prevalence of celiac disease: systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2018 Jun;16(6):823-36.http://www.ncbi.nlm.nih.gov/pubmed/29551598?tool=bestpractice.com 在临床实践中,女性在诊断患者中占了几乎三分之二。在乳糜泻患者中,难治性乳糜泻的发病率尚不完全清楚,但估计约为 1%。发病的第一个高峰期是在首次接触麸质后不久的婴儿期,第二个发病率更高的高峰期是在 30 岁至 50 岁左右。尽管在美国诊断时最常见的年龄大约为 40 岁,但乳糜泻可于任何年龄进行诊断。[7]Green PH. The many faces of celiac disease: clinical presentation of celiac disease in the adult population. Gastroenterology. 2005 Apr;128(4 Suppl 1):S74-8.http://www.ncbi.nlm.nih.gov/pubmed/15825130?tool=bestpractice.com[8]Green PH, Stavropoulos SN, Panagi SG, et al. Characteristics of adult celiac disease in the USA: results of a national survey. Am J Gastroenterol. 2001 Jan;96(1):126-31.http://www.ncbi.nlm.nih.gov/pubmed/11197241?tool=bestpractice.com
无症状性乳糜泻是指血清学和组织学检查表明存在乳糜泻的证据,但没有任何明显的症状、体征或营养元素的缺乏。目前尚不完全清楚真正无症状性乳糜泻的比例,但据估计至少占到此类患者的 20%。
难治性乳糜泻是属于无反应性乳糜泻类型中的一种特异性诊断,定义为在至少 6 个月的严格无麸质饮食后,临床症状和组织学异常持续存在,且无其他明显病因或淋巴瘤的表现。在乳糜泻患者中,难治性乳糜泻的发病率尚不完全清楚,但估计约为 1%。