医学
炎症性肠病
溃疡性结肠炎
四分位间距
常见可变免疫缺陷
原发性免疫缺陷
克罗恩病
维多利祖马布
内科学
胃肠病学
疾病
免疫学
抗体
作者
Ahmad Albshesh,Piotr Eder,Davide Giuseppe Ribaldone,Bas Oldenburg,Nanne K.H. de Boer,Gerassimos J. Mantzaris,Edoardo Savarino,Gabriele Dragoni,Roni Weisshof,Marie Truyens,Stefano Festa,Michel H. Maillard,Lena Capirchio,Rafał Filip,Eirini Theodoraki,Uri Kopylov
标识
DOI:10.1093/ecco-jcc/jjab124
摘要
Abstract Background Hypogammaglobulinaemia is a disorder characterized by low serum immunoglobulin levels and a high prevalence of gastrointestinal manifestations. In some cases, clinical and endoscopic features are indistinguishable from those of inflammatory bowel disease [IBD]. Methods This was a multicentre case series performed as a part of the European Crohn’s and Colitis Organisation [ECCO] Collaborative Network of Exceptionally Rare case reports [CONFER] project. Results This report includes 27 patients with primary hypogammaglobulinaemia and IBD-like features: 20 males and seven females, median age 45.6 years (interquartile range [IQR] 35.2–59). Crohn’s disease-like features were noted in 23 patients, and four patients had ulcerative colitis-like features. The diagnosis of hypogammaglobulinaemia preceded a diagnosis of IBD-like features in 20 patients [median of 7 years prior, IQR 2.6–20.6 years], and followed the appearance of IBD-like features in seven cases [median of 1 year after, IQR 0.45–5.6 years]. Hypogammaglobulinaemia aetiologies were common variable immunodeficiency [66.6%], agammaglobulinaemia [7.4%], selective IgA-deficiency [11.1%], Good’s syndrome [7.4%], IgG subclass deficiency with IgA deficiency [3.7%] and hyper-IgM [3.7%]. In addition to antibiotics and intravenous immunoglobulin [IVIG] for hypogammaglobulinaemia, 12 patients received IBD-related treatment including 5-aminosalicylate agents [two patients], corticosteroids [one patient], thiopurines [three patients], anti-tumour necrosis factor [four patients] and vedolizumab [two patients]. By the end of the follow-up (44.5 months [IQR 18–81]), 21/27 [77%] patients were in clinical remission. Conclusion This case series describes IBD-like features in patients with hypogammaglobulinaemia. The diagnosis of IBD-like features mainly occurred after that of hypogammaglobulinaemia, with successful recovery in the majority of cases after appropriate treatment.
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