成人中无乳糜泻抗体的绒毛萎缩(VA)的鉴别诊断包括血清阴性乳糜泻(CD)和与麸质无关的慢性肠病,即.非乳糜泻肠病(NCEs)。目前在这些肠病的命名和诊断标准上没有国际共识。在这项工作中,我们进行了Delphi程序来解决这种诊断和临床不确定性.
在第16届国际乳糜泻研讨会上,招募了一个由来自6个国家的13名胃肠病学家组成的国际工作组。巴黎,2019.在2019年9月至2021年7月之间,通过邮件调查进行了Delphi程序,以就在乳糜泻血清学阴性的VA的鉴别诊断中考虑哪些条件以及这些条件所需的临床诊断方法达成共识。采用了70%的协议门槛。
以VA和乳糜泻血清学阴性为特征的慢性肠病可归因于两种主要临床情况:呈阴性血清学的CD形式,其中还包括血清阴性CD和与IgA缺乏症相关的CD,和NCE,后者认识到不同的潜在病因。就帮助临床医生区分NCEs与血清阴性CD的NCEs诊断标准达成共识。尽管在成人血清阴性CD是VA和血清学阴性患者中最常见的病因,区分血清阴性CD和NCE是避免不必要的终身无麸质饮食的关键,治疗疾病特异性发病率和对比不良的长期结果。
本文描述了关于成人血清阴性CD和慢性NCE的定义和诊断标准的巴黎共识。
Differential diagnosis of villous atrophy (VA) without coeliac antibodies in adults includes seronegative coeliac disease (CD) and chronic enteropathies unrelated to gluten, ie. non-coeliac enteropathies (NCEs). There is currently no international
consensus on the nomenclature and diagnostic criteria for these enteropathies. In this work, a Delphi process was conducted to address this diagnostic and clinical uncertainty.
An international task force of 13 gastroenterologists from six countries was recruited at the 16th International Coeliac Disease Symposium, Paris, 2019. Between September 2019 and July 2021, a Delphi process was conducted through mail surveys to reach a
consensus on which conditions to consider in the differential diagnosis of VA with negative coeliac serology and the clinical diagnostic approaches required for these conditions. A 70% agreement threshold was adopted.
Chronic enteropathies characterised by VA and negative coeliac serology can be attributed to two main clinical scenarios: forms of CD presenting with negative serology, which also include seronegative CD and CD associated with IgA deficiency, and NCEs, with the latter recognising different underlying aetiologies. A
consensus was reached on the diagnostic criteria for NCEs assisting clinicians in differentiating NCEs from seronegative CD. Although in adults seronegative CD is the most common aetiology in patients with VA and negative serology, discriminating between seronegative CD and NCEs is key to avoid unnecessary lifelong gluten-free diet, treat disease-specific morbidity and contrast poor long-term outcomes.
This paper describes the Paris
consensus on the definitions and diagnostic criteria for seronegative CD and chronic NCEs in adults.