乳糜泻

应在诊断时将患者转诊给营养师，然后每年进行一次病情检查，以指导和监测他们依从无麸质饮食的情况。在开始无麸质饮食后，抗组织型转谷氨酰胺酶 IgA 抗体（immunoglobulin A-tissue transglutaminase, IgA-tTG）的正常化与黏膜愈合之间可能存在不一致性。[142]Silvester JA, Kurada S, Szwajcer A, et al. Tests for serum transglutaminase and endomysial antibodies do not detect most patients with celiac disease and persistent villous atrophy on gluten-free diets: a meta-analysis. Gastroenterology. 2017 Sep;153(3):689-701;e1.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738024/http://www.ncbi.nlm.nih.gov/pubmed/28545781?tool=bestpractice.com 黏膜完全恢复的时间有所不同；不到半数的乳糜泻患者在坚持无麸质饮食 1 年后十二指肠组织学表现恢复正常，成人比儿童黏膜愈合的可能性更小。[184]Szakács Z, Mátrai P, Hegyi P, et al. Younger age at diagnosis predisposes to mucosal recovery in celiac disease on a gluten-free diet: a meta-analysis. PLoS One. 2017 Nov 2;12(11):e0187526.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5695627/http://www.ncbi.nlm.nih.gov/pubmed/29095937?tool=bestpractice.com 症状并不能很好地预测黏膜炎症或恢复情况。[185]Mahadev S, Murray JA, Wu TT, et al. Factors associated with villus atrophy in symptomatic coeliac disease patients on a gluten-free diet. Aliment Pharmacol Ther. 2017 Apr;45(8):1084-93.https://onlinelibrary.wiley.com/doi/full/10.1111/apt.13988http://www.ncbi.nlm.nih.gov/pubmed/28220520?tool=bestpractice.com

如果患者在坚持无麸质饮食 1 年后出现临床和血清学缓解，可考虑在接下来的 2 年内每年进行一次随访，此后每 2 年一次。[34]Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019 Jun;7(5):583-613.https://journals.sagepub.com/doi/10.1177/2050640619844125?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub++0pubmed&http://www.ncbi.nlm.nih.gov/pubmed/31210940?tool=bestpractice.com​ 欧洲儿科胃肠病学、肝病学和营养学学会建议对确诊为乳糜泻的儿童进行定期随访，首次随访安排在诊断后 3-6 个月，随后每 6 个月随访一次，直至 tTG 水平恢复正常后每 12-24 个月随访一次。[186]Mearin ML, Agardh D, Antunes H, et al. ESPGHAN position paper on management and follow-up of children and adolescents with celiac disease. J Pediatr Gastroenterol Nutr. 2022 Sep 1;75(3):369-86.https://journals.lww.com/jpgn/Fulltext/2022/09000/ESPGHAN_Position_Paper_on_Management_and_Follow_up.29.aspxhttp://www.ncbi.nlm.nih.gov/pubmed/35758521?tool=bestpractice.com

应向患者开具口服补充剂处方，以治疗诊断时存在的任何营养缺乏症，并且应进行监测，直至这些缺乏症恢复正常。大多数营养缺乏症可通过无麸质饮食得到改善，但有些营养缺乏症需要更长时间（如缺铁、缺锌）。[187]Kreutz JM, Adriaanse MPM, van der Ploeg EMC, et al. Narrative review: nutrient deficiencies in adults and children with treated and untreated celiac disease. Nutrients. 2020 Feb 15;12(2):500.https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/32075276/http://www.ncbi.nlm.nih.gov/pubmed/32075276?tool=bestpractice.com[188]Stefanelli G, Viscido A, Longo S, et al. Persistent iron deficiency anemia in patients with celiac disease despite a gluten-free diet. Nutrients. 2020 Jul 22;12(8):2176.https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/32708019/http://www.ncbi.nlm.nih.gov/pubmed/32708019?tool=bestpractice.com​ 无麸质饮食期间可能会出现一些新的营养缺乏症，如维生素 B6 缺乏症。[187]Kreutz JM, Adriaanse MPM, van der Ploeg EMC, et al. Narrative review: nutrient deficiencies in adults and children with treated and untreated celiac disease. Nutrients. 2020 Feb 15;12(2):500.https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/32075276/http://www.ncbi.nlm.nih.gov/pubmed/32075276?tool=bestpractice.com

IgA-tTG 滴度通常在确诊后的第一年至少检查 3 次（3 个月、6 个月和 12 个月时），然后每年检查一次，作为饮食依从性的一个指征。[34]Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019 Jun;7(5):583-613.https://journals.sagepub.com/doi/10.1177/2050640619844125?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub++0pubmed&http://www.ncbi.nlm.nih.gov/pubmed/31210940?tool=bestpractice.com[143]Husby S, Bai JC. Follow-up of celiac disease. Gastroenterol Clin North Am. 2019 Mar;48(1):127-36.http://www.ncbi.nlm.nih.gov/pubmed/30711205?tool=bestpractice.com​ 大多数患者的 IgA-tTG 滴度应在 6-9 个月之内恢复正常，但有些患者（例如，严重黏膜萎缩、1 型糖尿病、诊断时滴度极高的儿童）可能需要 3 年以上的时间。[189]Mangione RA, Patel PN. Caring for patients with celiac disease: the role of the pharmacist. J Am Pharm Assoc (2003). 2008 Sep-Oct;48(5):e125-35.http://www.ncbi.nlm.nih.gov/pubmed/18826893?tool=bestpractice.com[190]Gidrewicz D, Trevenen CL, Lyon M, et al. Normalization time of celiac serology in children on a gluten-free diet. J Pediatr Gastroenterol Nutr. 2017 Mar;64(3):362-7. http://www.ncbi.nlm.nih.gov/pubmed/28231071?tool=bestpractice.com[191]Isaac DM, Rajani S, Yaskina M, et al. Antitissue transglutaminase normalization postdiagnosis in children with celiac disease. J Pediatr Gastroenterol Nutr. 2017 Aug;65(2):195-9.http://www.ncbi.nlm.nih.gov/pubmed/27906802?tool=bestpractice.com如果 IgA-tTG 滴度呈下降趋势，患者无症状，营养缺乏问题得到解决，则无需立即采取措施。

在确诊 2 年以后的患者中，即使 IgA-tTG 正常，也可以复查内窥镜，作为与患者共同决策过程的一部分确定是否存在麸质暴露，且应对并发症风险进行分层，因为癌症和骨病的风险主要与持续的绒毛萎缩有关。[73]Chaudrey KH. ACG guideline: diagnosis and management of celiac disease. Am J Gastroenterol. 2023;118(1):23.http://www.ncbi.nlm.nih.gov/pubmed/36602833?tool=bestpractice.com[142]Silvester JA, Kurada S, Szwajcer A, et al. Tests for serum transglutaminase and endomysial antibodies do not detect most patients with celiac disease and persistent villous atrophy on gluten-free diets: a meta-analysis. Gastroenterology. 2017 Sep;153(3):689-701;e1.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738024/http://www.ncbi.nlm.nih.gov/pubmed/28545781?tool=bestpractice.com[192]Rej A, Elli L, Sanders DS. Persisting villous atrophy and adherence in celiac disease: what does the patient want? What should a clinician advise? Am J Gastroenterol. 2021 May 1;116(5):946-48.http://www.ncbi.nlm.nih.gov/pubmed/33767095?tool=bestpractice.com

可在诊断时或坚持无麸质饮食 1 年后评估成人患者的骨密度。[34]Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019 Jun;7(5):583-613.https://journals.sagepub.com/doi/10.1177/2050640619844125?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub++0pubmed&http://www.ncbi.nlm.nih.gov/pubmed/31210940?tool=bestpractice.com[179]World Gastroenterology Organisation. WGO practice guideline: celiac disease. Jul 2016 [internet publication].http://www.worldgastroenterology.org/guidelines/global-guidelines/celiac-disease/celiac-disease-english​​​[180]Ludvigsson JF, Bai JC, Biagi F, et al. Diagnosis and management of adult coeliac disease: guidelines from the British Society of Gastroenterology. Gut. 2014 Aug;63(8):1210-28.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4112432/http://www.ncbi.nlm.nih.gov/pubmed/24917550?tool=bestpractice.com[193]West J, Logan RF, Card TR, et al. Fracture risk in people with celiac disease: a population-based cohort study. Gastroenterology. 2003 Aug;125(2):429-36.http://www.ncbi.nlm.nih.gov/pubmed/12891545?tool=bestpractice.com[194]Bai JC, Gonzalez D, Mautalen C, et al. Long-term effect of gluten restriction on bone mineral density of patients with coeliac disease. Aliment Pharmacol Ther. 1997 Feb;11(1):157-64.https://onlinelibrary.wiley.com/doi/epdf/10.1046/j.1365-2036.1997.112283000.xhttp://www.ncbi.nlm.nih.gov/pubmed/9042988?tool=bestpractice.com