Common variable immunodeficiency associated with Crohn-like disease: case report and literature review

  • Larissa Cristina Tavares de Castro Faculdade de Medicina de Marília
  • Zamir Calamita Faculdade de Medicina de Marília
Keywords: common variable immunodeficiency, Crohn disease, immunity, immune system diseases, inflammatory bowel disease.

Abstract

AIMS: To report the case of a patient diagnosed with common variable immunodeficiency and Crohn-like disease, describing the clinical picture, the diagnostic investigation process, the therapeutic approaches and the clinical outcome of the patient. To carry out a literature review of case reports addressing patients with the common variable immunodeficiency and Crohn-like disease association. Emphasize the importance of early diagnosis and treatment.

CASE DESCRIPTION: A male patient was diagnosed with common variable immunodeficiency at nine years of age and with non-specific inflammatory bowel disease at 10 years, after colonoscopy and colonic biopsy indicated moderate lymphoplasmacytic, eosinophilic infiltrate and some neutrophils in lamina propria, with absence of granulomas. At age 14, the diagnosis of Crohn-like disease was confirmed by specialist after correlation between clinical history and complementary exams. Currently with 18 years of age, the patient is under treatment with intravenous human immunoglobulin, infliximab and azathioprine, with stabilization of the clinical picture.

CONCLUSIONS: The literature review identified 11 case reports on the association between common variable immunodeficiency and Crohn-like disease, with no Brazilian studies, which highlights the rarity of such association. In this report, the patient received extensive and empirical treatments due to the difficulty in reaching a specific diagnosis, which was only performed at age 14, when more targeted and individualized treatment was started. Although currently stable, the patient presented several complications during the diagnostic investigation, which emphasizes the importance of early diagnosis and more precise treatment, targeted to meet the health needs of these patients.

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Author Biographies

Larissa Cristina Tavares de Castro, Faculdade de Medicina de Marília
Graduanda de Medicina na Faculdade de Medicina de Marília
Zamir Calamita, Faculdade de Medicina de Marília
Doutor Chefe da Disciplina de Alergia e Imunopatologia Clínica da Faculdade de Medicina de Marília

References

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(12):1800-12.

https://doi.org/10.1097/PAS.0b013e3180cab60c

Tegtmeyer D, Seidl M, Gerner P, Baumann U, Klemann C. Inflammatory bowel disease caused by primary immunodeficiencies - clinical presentations, review of literature, and proposal of a rational diagnostic algorithm. Pediatr Allergy Immunol. 2017;28(5):412-29.

https://doi.org/10.1111/pai.12734

Akazawa Y, Takeshima F, Yajima H, Imanishi D, Kanda T, Matsushima K, Minami H, Yamaguchi N, Ohnita K, Isomoto H, Hayashi T, Nakashima M, Nakao K. Infliximab therapy for Crohn’s-like disease in common variable immunodeficiency complicated by massive intestinal hemorrhage: a case report. BMC Res Notes. 2014;7:382.

https://doi.org/10.1186/1756-0500-7-382

Vázquez-Morón JM, Pallarés-Manrique H, Martín-Suárez IJ, Benítez-Rodríguez B, Ramos-Lora M. Crohn’s-like disease in a patient with common variable immunodeficiency treated with azathioprine and adalimumab. Rev Esp Enferm Dig. 2013;105(5):299-302.

https://doi.org/10.4321/S1130-01082013000500010

Salzer U, Warnatz K, Peter HH. Common variable immunodeficiency - an update. Arthritis Res Ther. 2012;14(5):223.

https://doi.org/10.1186/ar4032

Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J. Medicina interna de Harrison. 18ª ed. Porto Alegre: AMGH Editora Ltda; 2013. p. 2477-95.

Greer RL, Morgun A, Shulzhenko N. Bridging immunity and lipid metabolism by gut microbiota. J Allergy Clin Immunol. 2013;132(2):25362.

https://doi.org/10.1016/j.jaci.2013.06.025

Packwood K, Drewe E, Staples E, Webster D, Witte T, Litzman J, Egner W, Sargur R, Sewell W, Lopez-Granados E, Seneviratne SL, Powell RJ, Ferry BL, Chapel HM. NOD2 polymorphisms in clinical phenotypes of common variable immunodeficiency disorders. Clin Exp Immunol. 2010;161(3):536-41.

https://doi.org/10.1111/j.1365-2249.2010.04216.x

Salda-a DC, Rubio IS. Immunodeficiencies and autoimmune diseases: common variable immunodeficiency and Crohnlike. Rev Esp Enferm Dig. 2016;108(8):520-3.

https://doi.org/10.17235/reed.2015.3872/2015

Mannon PJ, Fuss IJ, Dill S, Friend J, Groden C, Hornung R, Yang Z, Yi C, Quezado M, Brown M, Strober W. Excess IL-12 but not IL-23 accompanies the inflammatory bowel disease associated with common variable immunodeficiency. Gastroenterology. 2006;131(3):748-56.

https://doi.org/10.1053/j.gastro.2006.06.022

Bosworth BP, Sanders A, Maltz C. Common variable immunodeficiency masquerading as Crohn´s ileocolitis. Inflamm Bowel Dis. 2006;12(2):151-2.

https://doi.org/10.1097/01.MIB.0000200344.61707.9c

Sanges M, Spadaro G, Miniero M, Mattera D, Sollazzo R, D’Armiento FP, De Palma GD, Pecoraro A, Borrelli F, Genovese A, D’Arienzo A. Efficacy of subcutaneous immunoglobulins in primary immunodeficiency with Crohn’s-like phenotype: report of a case. Eur Rev Med Pharmacol Sci. 2015;19(14):2641-5.

Mudter J, Wirtz S, Weigmann B, Tiede I, Tubbe I, Kiesslich R, Galle PR, Lehr HA, Neurath MF. Crohn’s-like colitis in a patient with immunodeficiency associated with a defect in expression of inducible costimulator. Dig Dis Sci. 2006;51(4):711-7.

https://doi.org/10.1007/s10620-006-3196-x

Strauss RG, Ghishan F, Mitros F, Ebensberger JR, Kisker CT, Tannous R, Younoszai MK. Rectosigmoidal colitis in common variable immunodeficiency disease. Dig Dis Sci.1980;25(10):798-801.

https://doi.org/10.1007/BF01345303

Ünal B, Başsorgun Cİ, Gönülcü SC, Uçar A, Çelik F, Elpek GÖ. Cytomegalovirus colitis with common variable immunodeficiency and Crohn’s disease. Case Rep Med. 2015;2015:348204.

https://doi.org/10.1155/2015/348204

Salado CT, Carnerero EL, Moreno AP, Ramírez MC, Miguez AA, Justiniano JH, Galán JM. Tratamiento con inmunosupresores en paciente con enfermedad de Crohn e inmunodeficiencia común variable. Gastroenterol Hepatol. 2015;38(9):544-5.

https://doi.org/10.1016/j.gastrohep.2014.09.008

Tuna Y, Koçak E, Köklü S. Common variable immunodeficiency associated with Crohn´s disease. Acta Clin Belg. 2011;66(1):75-6.

https://doi.org/10.1179/ACB.66.1.2062524

Nos P, Bastida G, Beltran B, Aguas M, Ponce J. Crohn´s disease in common variable immunodeficiency: treatment with antitumor necrosis factor alpha. Am J Gastroenterol. 2006;101(9):2165-6.

https://doi.org/10.1111/j.1572-0241.2006.00763_5.x

Published
2019-01-17
How to Cite
de Castro, L. C. T., & Calamita, Z. (2019). Common variable immunodeficiency associated with Crohn-like disease: case report and literature review. Scientia Medica, 29(1), e32159. https://doi.org/10.15448/1980-6108.2019.1.32159
Section
Case Reports