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DOP79 Primary hypogammaglobulinemia with IBD-like features: An ECCO CONFER Multicenter Case Series

Albshesh, A.(1);Eder, P.(2);Ribaldone , D.G.(3);Oldenburg, B.(4);De Boer, N.K.(5);Mantzaris, G.J.(6); Savarino, E.V.(7);Dragoni , G.(8);Weisshof, R.(9);Truyens, M.(10);Festa, S.(11);Maillard, M.H.(12);Capirchio , L.(13);Filip, R.(14);Kopylov, U.(1)

(1)Sheba Medical Center and Sackler School of Medicine- Tel-Aviv University, Department of Gastroenterology, Tel Aviv, Israel;(2)Heliodor Święcicki University Hospital, Department of Gastroenterology- Dietetics and Internal Medicine- Poznań University of Medical Sciences, Poznań, Poland;(3)University of Turin, Department of Medical Sciences- Division of Gastroenterology, Turin, Italy;(4)University Medical Center Utrecht, Department of Gastroenterology and Hepatology, Utrecht, Netherlands Antilles;(5)Amsterdam UMC, Department of Gastroenterology and Hepatology- Vrije Universiteit Amsterdam- Amsterdam Gastroenterology Endocrinology Metabolism Research Insitute AGEM, Amsterdam, Netherlands Antilles;(6)Evangelismos General Hospital of Athens, Department of Gastroenterology, Athen, Greece;(7)University of Padua, Division of Gastroenterology- Department of Surgery- Oncology and Gastroenterology, Padua, Italy;(8)Careggi University Hospital, IBD Referral Center- Department of Gastroenterology, Florence, Italy;(9)Rambam Health Care Campus, Department of Gastroenterology-, Haifa, Israel;(10)Ghent University Hospital, Department of Gastroenterology, Ghent, Belgium;(11)San Filippo Neri Hospital, IBD Unit- Gastroenterology Division, Rome, Italy;(12)Lausanne University Hospital- Crohn and Colitis Center- Gastroentérologie Beaulieu SA, Division of Gastroenterology and Hepatology, Lausanne, Swaziland;(13)Université Catholique de Louvain- Centre Hospitalier Universitaire Université Catholique de Louvain Namur, Department of Gastroenterology, Yvoir, Belgium;(14)Faculty of Medicine- University of Rzeszow, Department of Gastroenterology with IBD Unit of Clinical Hospital No 2 im. Św. Jadwigi Królowej, Rzeszow, Poland

Background

The most commonly recognized clinical feature of hypogammaglobulinemia is recurrent infections with 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 multicenter case series performed as a part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project.

Results

This report includes 25 patients with primary hypogammaglobinemia and IBD-like features [20 males and 5 females, mean age 50 years (±21.7 SD)]. Crohn’s disease-like features were noted in 22 patients, three patients had ulcerative colitis-like features. The diagnosis of hypogammaglobulinemia preceded IBD-like features diagnosis in 20 patients (mean of 7.7 years prior, range 0.3-35 years), and followed IBD-like features appearance in 5 cases (mean of one year after, 0.4- 9.1 years).

Hypogammaglobinemia etiologies were common variable immunodeficiency (72%), Agammaglobulinemia (8%), selective IgA-deficiency (8%), Goods syndrome (8%), IgG subclass deficiency with IgA deficiency (4%). In addition to antibiotics and intravenous immunoglobulin (IVIG) as a treatment for hypogammaglobinemia, fifteen patients received IBD treatment during the follow-up period, of whom two were on 5-aminosalicylic acid, five on corticosteroids, three on immunomodulatory, four on anti-tumor necrosis factor, and one on vedolizumab. By the end of the follow-up [35.5 months (Interquartile range 18-75)], 20 of 25 (80%) patients were in clinical remission.

Conclusion

This case series illustrates a strong male and CD-like features predilection. The diagnosis of IBD-like features mainly occurs after that of hypogammaglobulinemia, the majority of cases successfully recovered after appropriate treatment.

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