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Association between common variable immunodeficiency and collagenous infiltrative disorders of the gastrointestinal tract: A series of four patients

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Abstract

Hypogammaglobulinemia/common variable immunodeficiency (CVID) may lead to disruption of the gut mucosal immune barrier. Collagenous infiltrative disorders of the intestinal tract (colitis, gastritis, sprue) constitute a relatively new spectrum of gastrointestinal disorders. Our aims were (1) to determine the association between immunoglobulin deficiency state like CVID and collagenous infiltrative disorders of the gut and (2) to study the clinic-pathologic characteristics and treatment outcomes in these patients. A retrospective search was conducted to identify cases with concurrence of these two conditions at an academic center from 2007 to 2013. Four such patients were identified from our database: three with collagenous colitis and one with collagenous gastritis. All patients with collagenous colitis had normal colonic mucosa while the patient with collagenous gastritis had nodular gastric mucosa. Only one patient out of four had decreased plasma cells in the submucosa as expected in low immunoglobulin states. All patients had improvement in their symptoms on immunoglobulin therapy with considerable remission on budesonide. Literature search revealed reporting of four similar patients. In conclusion, (1) the association between collagenous infiltrative disorders of the gut and CVID and its prompt response to immunoglobulins with effective maintenance with budesonide are novel findings. Our study also shows that the presence of plasma cells should not rule out the possibility of CVID. (2) In patients with chronic diarrhea, hypogammaglobulinemia and collagenous colitis/sprue should be considered for the available effective treatments such as immunoglobulins and budesonide.

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References

  1. Rosen F, Cooper M, Wedgwood R. The primary immunodeficiencies. N Engl J Med. 1995;333:431–40.

    Article  CAS  PubMed  Google Scholar 

  2. Elnachef N, McMorris M, Chey WD. Successful treatment of common variable immunodeficiency disorder-associated diarrhea with budesonide: a case report. Am J Gastroenterol. 2007;102:1322–5.

    Article  PubMed  Google Scholar 

  3. Lai Ping So A, Mayer L. Gastrointestinal manifestations of primary immunodeficiency disorders. Semin Gastrointest Dis. 1997;8:22–32.

    CAS  PubMed  Google Scholar 

  4. Kalha I, Sellin JH. Common variable immunodeficiency and the gastrointestinal tract. Curr Gastroenterol Rep. 2004;6:377–83.

    Article  PubMed  Google Scholar 

  5. Ghoshal UC, Goel A, Ghoshal U, Jain M, Misra A, Choudhuri G. Chronic diarrhea and malabsorption due to hypogammaglobulinemia: a report on twelve patients. Indian J Gastroenterol. 2011;30:170–4.

    Article  PubMed  Google Scholar 

  6. Liszka L, Woszczyk D, Pajak J. Histopathological diagnosis of microscopic colitis. J Gastroenterol Hepatol. 2006;21:792–7.

    Article  PubMed  Google Scholar 

  7. Lagorce-Pages C, Fabiani B, Bouvier R, Scoazec JY, Durand L, Flejou JF. Collagenous gastritis: a report of six cases. Am J Surg Pathol. 2001;25:1174–9.

    Article  CAS  PubMed  Google Scholar 

  8. Byrne MF, Royston D, Patchett SE. Association of common variable immunodeficiency and atypical collagenous colitis. Eur J Gastroenterol Hepatol. 2003;15:1051–3.

    Article  PubMed  Google Scholar 

  9. Yong PFK, Li H, Chung-Faye G, Ibrahim MA. Collagenous colitis in a patient with common variable immunodeficiency. J Investig Allergol Clin Immunol. 2008;18:482–3.

    CAS  PubMed  Google Scholar 

  10. Pariente EA, Chaumette MT, Maître F, Delchier JC, Soulé JC, Bader JP. Collagenous colitis, IgA deficiency, Basedow’s disease and atrophic gastritis. Gastroenterol Clin Biol. 1985;9:738–41.

    CAS  PubMed  Google Scholar 

  11. Navarro-Llavat M, Domènech E, Masnou H, et al. Collagenous duodeno-ileo-colitis with transient IgG deficiency preceded by Yersinia enterocolitica intestinal infection: case report and review of literature. Gastroenterol Hepatol. 2007;30:219–21.

    Article  PubMed  Google Scholar 

  12. Daniels JA, Lederman HM, Maitra A, Montgomery EA. Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. Am J Surg Pathol. 2007;31:1800–12.

    Article  PubMed  Google Scholar 

  13. Janeway CA, Apt L, Gitlin D. Agammaglobulinemia. Trand Assoc Am Physicians. 1953;66:200–2.

    CAS  Google Scholar 

  14. Washington K, Stenzel TT, Buckley RH, Gottfried MR. Gastrointestinal pathology in patients with common variable immunodeficiency and X-linked agammaglobulinemia. Am J Surg Pathol. 1996;20:1240–52.

    Article  CAS  PubMed  Google Scholar 

  15. Mandaliya R, DiMarino AJ, Abraham S, Burkart A, Cohen S. Collagenous gastritis a rare disorder in search of a disease. Gastroenterol Res. 2013;6:139–44.

    Google Scholar 

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Funding sources

Thomas Jefferson University Hospitals, National Institutes of Diabetes and Digestive and Kidney Diseases Grant DK- 35385 to Dr. Satish Rattan.

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Correspondence to Rohan Mandaliya.

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RM, ALB, AJD, SR, and SC declare that they have no conflict of interest.

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The study was performed in a manner to conform to the Helsinki Declaration of 1975 as revised in 2000 and 2008 concerning Human and Animal Rights, and the authors followed the policy concerning informed consent as shown on Springer.com.

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Mandaliya, R., Burkart, A.L., DiMarino, A.J. et al. Association between common variable immunodeficiency and collagenous infiltrative disorders of the gastrointestinal tract: A series of four patients. Indian J Gastroenterol 35, 133–138 (2016). https://doi.org/10.1007/s12664-016-0633-4

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  • DOI: https://doi.org/10.1007/s12664-016-0633-4

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