Gastrointestinal manifestations in patient with Common Variable Immunodeficiency Syndrome (CVID): A Case Report

Gastrointestinal manifestations in patient with Common Variable Immunodeficiency Syndrome (CVID): A Case Report

B. Christopher1, MS Ismail1, M. Kirca2, D. McNamara1

1Department of Gastroenterology, Tallaght University Hospital & Department of Clinical Medicine, Trinity College Dublin, Dublin Ireland; 2Department of Gastroenterology, Mullingar General Hospital, Mullingar, Ireland

Open Journal of Gastroenterology and Hepatology

A 57 yo male with a background history of common variable Immunodeficiency syndrome (CVID) on Immunoglobulin Infusion (Kiovig) 40mg three weekly was referred for investigation of diarrhoea and follow up from previous history of colonic polyps.
Colonoscopy showed an irregular looking ileocaecal valve (ICV) with an adjacent flat polyp (Paris IIa). Biopsies showed low grade dysplasia. There was also a duodenal polyp noted on gastroscopy and biopsy again showed low grade dysplasia. In light of the findings of upper and lower gastrointestinal tract polyps, a small bowel capsule endoscopy (SBCE) was arranged. This showed an irregular area of mucosa in the proximal small bowel with significant ulceration and inflammation (Figure 1). There were also multiple scattered lymphagiectasias and lymphoid hyperplasia in the distal small bowel (Figure 2).
Anterograde double ballon enteroscopy (ADBE) was subsequently performed to the distal jejunum about 8 weeks after the SBCE. The duodenal polyp seen at gastroscopy was visualised during ADBE (Figure 3). In addition, two diminutive (<3mm) jejunal sessile polyps were encountered and excised (Figure 4). There was no evidence of ulcerative enteritis as seen on SBCE. The enteritis features initially visualized on the SBCE was thought to be a transient phenomenon possibly related to his CVID background.
However, for completion, the distal point of enteroscopy insertion was marked and a repeat SBCE performed immediately after recovery from the ADBE. This again showed the known duodenal polyp as well as 2 further diminutive small bowel polyps and minimal distal patchy enteritis only, with complete resolution of the previously observed ulcerative jejunitis. The jejunal polyps’ histology was reviewed at a histology multidisciplinary meeting, there were no adenomatous or hamartomatous features and biopsies were thought to have non-specific changes only.
The patient was referred for polyp resection and further follow up and surveillance will be arranged based on histology.

Keywords: Gastrointestinal manifestations, Common Variable Immunodeficiency Syndrome, CVID, Case Report

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How to cite this article:
B.Christopher, MS Ismail, M.Kirca, D.McNamara. Gastrointestinal manifestations in patient with Common Variable Immunodeficiency Syndrome (CVID): A Case Report.Open Journal of Gastroenterology and Hepatology, 2019, 2:18. DOI: 10.28933/ojgh-2019-10-1805


1. Bonilla FA, Barlan I, Chapel H, Costa-Carvalho BT, Cunningham-Rundles C, de la Morena MT, et al. ICON: Common Variable Immunodeficiency Disorders. J allergy Clin Immunol Pract. 2015
2. Picard C, Al-Herz W, Bousfiha A, Casanova J-L, Chatila T, Conley ME, et al. Primary Immunodeficiency Diseases: an Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015. J Clin Immunol. 2015
3. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119:1650–7
4. Song J,Lleo A, Yang GX, Zhang W, Bowlus CL, Gershwin ME, Leung PSC. Clin Rev Allergy Immunol. 2017 Aug 7. Common Variable Immunodeficiency and Liver Involvement.
5. Mathieu Uzzan, Huaibin M. Ko, Saurabh Mehandru, Charlotte Cunningham-Rundles. Gastrointestinal Disorders Associated with Common Variable Immune Deficiency (CVID) and Chronic Granulomatous Disease (CGD). Curr Gastroenterol Rep. 2016 Apr; 18(4): 17.
6. Malamut G, Verkarre V, Suarez F, Viallard J-F, Lascaux A-S, Cosnes J, et al. The enteropathy associated with common variable immunodeficiency: the delineated frontiers with celiac disease. Am J Gastroenterol. 2010;105:2262–75.
7. Gathmann B, Mahlaoui N, Gérard L, Oksenhendler E, Warnatz K, Schulze I, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134:116–26. e11
8. Cunningham-Rundles C. The many faces of common variable immunodeficiency. Hematology Am Soc Hematol Educ Program. 2012;2012:301–5
9. Dhalla F, Silva SP, Lucas M, Travis S, Chapel H. Review of gastric cancer risk factors in patients with common variable immunodeficiency disorders, resulting in a proposal for a surveillance programme. Clin Exp Immunol. 2011;165(1):1–7.

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