Advanced search
1 file | 1.02 MB Add to list

Primary hypogammaglobulinaemia with inflammatory bowel disease-like features : an ECCO CONFER multicentre case series

(2022) JOURNAL OF CROHNS & COLITIS. 16(1). p.91-97
Author
Organization
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.
Keywords
Gastroenterology, General Medicine, Primary hypogammaglobulinaemia, immunodeficiency, inflammatory bowel disease, IBD-like features, COMMON VARIABLE IMMUNODEFICIENCY, GASTROINTESTINAL MANIFESTATIONS, IMMUNE-DEFICIENCY, GOODS-SYNDROME, IGA, COMPLICATIONS, PATHOGENESIS, DIAGNOSIS, PATHOLOGY, DIARRHEA

Downloads

  • Albshesh 2021 Primary hypogammaglobulinemia in IBD.pdf
    • full text (Accepted manuscript)
    • |
    • open access
    • |
    • PDF
    • |
    • 1.02 MB

Citation

Please use this url to cite or link to this publication:

MLA
Albshesh, Ahmad, et al. “Primary Hypogammaglobulinaemia with Inflammatory Bowel Disease-like Features : An ECCO CONFER Multicentre Case Series.” JOURNAL OF CROHNS & COLITIS, vol. 16, no. 1, 2022, pp. 91–97, doi:10.1093/ecco-jcc/jjab124.
APA
Albshesh, A., Eder, P., Ribaldone, D. G., Oldenburg, B., de Boer, N. K., Mantzaris, G. J., … Kopylov, U. (2022). Primary hypogammaglobulinaemia with inflammatory bowel disease-like features : an ECCO CONFER multicentre case series. JOURNAL OF CROHNS & COLITIS, 16(1), 91–97. https://doi.org/10.1093/ecco-jcc/jjab124
Chicago author-date
Albshesh, Ahmad, Piotr Eder, Davide Giuseppe Ribaldone, Bas Oldenburg, Nanne K de Boer, Gerassimos J Mantzaris, Edoardo Vincenzo Savarino, et al. 2022. “Primary Hypogammaglobulinaemia with Inflammatory Bowel Disease-like Features : An ECCO CONFER Multicentre Case Series.” JOURNAL OF CROHNS & COLITIS 16 (1): 91–97. https://doi.org/10.1093/ecco-jcc/jjab124.
Chicago author-date (all authors)
Albshesh, Ahmad, Piotr Eder, Davide Giuseppe Ribaldone, Bas Oldenburg, Nanne K de Boer, Gerassimos J Mantzaris, Edoardo Vincenzo Savarino, Gabriele Dragoni, Roni Weisshof, Marie Truyens, Stefano Festa, Michel H Maillard, Lena Capirchio, Rafal Filip, Eirini Theodoraki, and Uri Kopylov. 2022. “Primary Hypogammaglobulinaemia with Inflammatory Bowel Disease-like Features : An ECCO CONFER Multicentre Case Series.” JOURNAL OF CROHNS & COLITIS 16 (1): 91–97. doi:10.1093/ecco-jcc/jjab124.
Vancouver
1.
Albshesh A, Eder P, Ribaldone DG, Oldenburg B, de Boer NK, Mantzaris GJ, et al. Primary hypogammaglobulinaemia with inflammatory bowel disease-like features : an ECCO CONFER multicentre case series. JOURNAL OF CROHNS & COLITIS. 2022;16(1):91–7.
IEEE
[1]
A. Albshesh et al., “Primary hypogammaglobulinaemia with inflammatory bowel disease-like features : an ECCO CONFER multicentre case series,” JOURNAL OF CROHNS & COLITIS, vol. 16, no. 1, pp. 91–97, 2022.
@article{8735667,
  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.}},
  author       = {{Albshesh, Ahmad and Eder, Piotr and Ribaldone, Davide Giuseppe and Oldenburg, Bas and de Boer, Nanne K and Mantzaris, Gerassimos J and Savarino, Edoardo Vincenzo and Dragoni, Gabriele and Weisshof, Roni and Truyens, Marie and Festa, Stefano and Maillard, Michel H and Capirchio, Lena and Filip, Rafal and Theodoraki, Eirini and Kopylov, Uri}},
  issn         = {{1873-9946}},
  journal      = {{JOURNAL OF CROHNS & COLITIS}},
  keywords     = {{Gastroenterology,General Medicine,Primary hypogammaglobulinaemia,immunodeficiency,inflammatory bowel disease,IBD-like features,COMMON VARIABLE IMMUNODEFICIENCY,GASTROINTESTINAL MANIFESTATIONS,IMMUNE-DEFICIENCY,GOODS-SYNDROME,IGA,COMPLICATIONS,PATHOGENESIS,DIAGNOSIS,PATHOLOGY,DIARRHEA}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{91--97}},
  title        = {{Primary hypogammaglobulinaemia with inflammatory bowel disease-like features : an ECCO CONFER multicentre case series}},
  url          = {{http://doi.org/10.1093/ecco-jcc/jjab124}},
  volume       = {{16}},
  year         = {{2022}},
}

Altmetric
View in Altmetric
Web of Science
Times cited: