Sara Şebnem Kılıç, Tanju Özkan, Yasin Karalı, Taner Özgür, Şükrü Çekiç

Vedolizumab treatment in a patient with X-linked agammaglobulinemia, is it safe and efficient?

The loss of inflammatory regulation resulting from the absence ofB-lymphocytes leads to a risk for autoimmune and autoinflammatorycomplications. There is no data on the use of Vedolizumab in patients withX-linked agammaglobulinemia (XLA) as well as children with another primaryimmunodeficiency (PID) diseases. A 4-year-old boy was admitted to our clinicwith a history of recurrent respiratory tract infections. He was diagnosedwith XLA based on extremely low immunoglobulins, very low level of B cells,genetic mutation of BTK gene, and family history. At the age of 8, he sufferedfrom intermittent fever attacks, abdominal pain, weakness, oral aft, and weightloss. His clinical and laboratory features were consistent with inflammatorybowel disease. Histopathological examination of the biopsy material obtainedfrom terminal ileum, colon and cecum showed Crohn’s disease. Initially, hewas treated with prednisolone and infliximab. Because of the lack of response,infliximab treatment was switched to adalimumab. Terminal ileum wasresected to relieve obstruction complication. Although he had been treatedwith adalimumab, a significant improvement was not observed. Vedolizumab(Entyvio™), a humanized monoclonal antibody α4β7 integrin-receptorantagonist, was commenced. After treatment with vedolizumab, his feverand abdominal pain attacks reduced, his total daily calorie intake increasedand weight gain improved. He began to walk again and continued his schooleducation properly. No side effects were observed in 18 months. This is thefirst immunocompromised child treated with vedolizumab. The symptoms ofthe patient receded and no side effect were seen during the treatment.

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1. Aneja A, Chhabra A. Bruton Agammaglobulinemia. [Updated 2017 Oct 9]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2018 Jan. Available from: http:// europepmc.org/books/NBK448170;jsessionid= CC9B9257254CF5E80DF7FF32D091BA
2. Hernandez-Trujillo VP, Scalchunes C, CunninghamRundles C, et al. Autoimmunity and inflammation in X-linked agammaglobulinemia. J Clin Immunol 2014; 34: 627-632.
3. Barmettler S, Otani IM, Minhas J, et al. Gastrointestinal manifestations in X-linked agammaglobulinemia. J Clin Immunol 2017; 37: 287-294.
4. Walter JE, Farmer JR, Foldvari Z, Torgerson TR, Cooper MA. Mechanism-based strategies for the management of autoimmunity and immune dysregulation in primary immunodeficiencies. J Allergy Clin Immunol Pract 2016; 4: 1089-1100.
5. Hyams J, Markowitz J, Otley A, et al; Pediatric Inflammatory Bowel Disease Collaborative Research Group. Evaluation of the pediatric crohn disease activity index: a prospective multicenter experience. J Pediatr Gastroenterol Nutr 2005; 41: 416-421.
6. Agarwal S, Mayer L. Pathogenesis and treatment of gastrointestinal disease in antibody deficiency syndromes. J Allergy Clin Immunol 2009; 124: 658- 664.
7. 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: 1240-1252.
8. Davey PT, Tan CJ, Gardiner K. The use of infliximab in X-linked agammaglobulinaemia associated enteropathy. Ann R Coll Surg Engl 2014; 96: e5-e6.
9. Robles-Marhuenda A, Cobo-Ibáñez T, Ríos-Blanco J, Arnalich-Fernández F. AB0927 biological drugs use in primary immunodeficiencies. Ann Rheum Dis 2016; 75(Suppl 2): 1218.
10. Fedyk ER, Wyant T, Yang LL, et al. Exclusive antagonism of the α4β7 integrin by vedolizumab confirms the gut-selectivity of this pathway in primates. Inflamm Bowel Dis 2012; 18: 2107-2119.
11. Boland BS, Riedl MA, Valasek MA, Crowe SE, Sandborn WJ. Vedolizumab in patients with common variable immune deficiency (CVID) and gut inflammation. Am J Gastroenterol 2017; 112: 1621.