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Background Common variable immune deficiency (CVID) can be associated with autoimmune manifestations including enteric inflammation and diarrhea. Systemic immunosuppression used in patients with inflammatory bowel disease (IBD) may be associated with increased risk of infection in CVID. Aims We report a patient with CVID associated intestinal enteropathy who underwent clinical, endoscopic and histological improvement after treatment with vedolizumab (IgG1 monoclonal antibody to α4β7 integrin), as well as a concurrent systematic review (SR) of the literature. Methods Case report and systematic literature review. We searched EMBASE, Medline, Cochrane CENTRAL, clinialtrials.gov, and the International Clinical Trials Registry Platform without language restriction using key words to identify patients with CVID associated enteropathy treated with vedolizumab. Clinical, endoscopic and histological outcomes were extracted and safety data. Results A 32-year-old male presented with weight loss, anemia and hypoalbuminemia on a background of chronic diarrhea and recurrent sinus infections. Serology and genetic testing was not consistent with celiac disease. Pan-endoscopy showed complete villous atrophy in the duodenum and biopsies showed and intraepithelial lymphocytosis (IEL) in the duodenum and terminal ileum. CT abdomen showed pan-enteritis with extensive mesenteric lymphadenopathy which were reactive on lymph node excision, as well as bronchiectasis. Pneumococcal vaccination challenge to assess humoral response confirmed CVID. After suboptimal response with steroids and mercaptopurine, treatment was initiated with vedolizumab 300mg IV at weeks 0, 2, and 6, then 8 weekly. This led to normalization of stool frequency, weight gain as well as endoscopic and histological resolution within 6 months of treatment. The SR yielded 101 studies of which 3 case series were identified reporting a total of 7 patients with CVID. In 5 cases there was clinical improvement, 4 reported endoscopic improvement and 3 patients had histologic improvement. No safety concerns associated with vedolizumab were reported. Conclusions Vedolizumab, a selective leucocyte inhibitor to the gut, was able to induce either clinical, endoscopic or histological improvement in 8 published cases in the worldwide literature. Prospective studies are needed to determine whether this treatment could be included in the therapeutic armamentarium for this orphan indication. Funding Agencies None

A234 CLINICAL, ENDOSCOPIC AND HISTOLOGICAL IMPROVEMENT IN COMMON VARIABLE IMMUNODEFICIENCY DISEASE ASSOCIATED ENTEROPATHY WITH VEDOLIZUMAB