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Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency, frequently presenting as recurrent upper respiratory infections. A portion of these patients develop non-specific gastrointestinal symptoms with a smaller subset developing CVID enteropathy, characterized by chronic diarrhea with absence of plasma cells and aggregates of T cells on intestinal pathology. We describe two cases of steroid refractory CVID enteropathy who were started on Vedolizumab (VDZ) with clinical remission. CASE 1 A 26 year-old female with primarily sinopulmonary CVID on immunoglobulin (IVIG) who developed profuse diarrhea. Infectious work-up and celiac biopsies were negative. Shortly after, colonoscopy with biopsies showing decreased plasma cells consistent with CVID enteropathy. She was started on budesonide with resolution of symptoms. After two months was weaned to 6 mg and began to have daily diarrhea. She was weaned to 3 mg budesonide daily and began to have more frequent diarrhea, bloating, weight loss. Later, budesonide was increased to 6 mg with no response then maximized to 9 mg daily. She was treated with standard induction and maintenance dosing of VDZ 300 with symptomatic remission. Budesonide was tapered off after completing third dose of vedolizumab. The patient has not had any more weight loss and stools are more formed. Her symptoms are abated until days prior to her next infusion; thus, VDZ interval was changed to every 7 weeks. She has not yet had colonoscopy to assess for mucosal healing but has had discontinuation of glucocorticoids. There were no adverse infectious events. CASE 2 A 28-year-old female with a known history of CVID enteropathy presented to GI clinic for refractory daily diarrhea despite chronic glucocorticoid therapy. She was diagnosed with CVID in 1998 and takes subcutaneous IVIG regularly. Colonoscopy showed mild chronic focally active ileitis, chronic active colitis. She started budesonide 9 mg; however, developed striae and weaned off budesonide. Subsequently, she was trialed on tacrolimus 0.1 mg/kg BID but suffered body aches and contracted COVID-19. She successively had worsening of stool frequency. Colonoscopy revealed ileum had loss of villous architecture and histologic evidence of CVID. She was treated with standard induction and maintenance dose of VDZ 300 mg with symptomatic remission. Symptoms dramatically improved and repeat endoscopy demonstrated no colitis. There were no adverse infectious events. DISCUSSION VDZ is an attractive therapy as the gut selective mechanism of action lowers risk of opportunistic infections and has emerged as an appealing therapeutic choice for this specific population. Some case reports have been mixed efficacy of inducing remission with VDZ; however, we report 2 cases of steroid resistant CVID enteropathy with clinical remission after induction with VDZ.

VEDOLIZUMAB AS TREATMENT FOR COMMON VARIABLE IMMUNE DEFICIENCY (CVID): A CASE SERIES AND BRIEF REVIEW OF THE LITERATURE