The Natural History of Inflammatory Bowel Disease in Adults With Common Variable Immunodeficiency: A Case Series From a Single US Tertiary Care Center

Background Common variable immunodeficiency (CVID) predisposes patients to inflammatory bowel disease (IBD). There are limited studies characterizing adults with concomitant CVID and IBD (CVID-IBD).Methods Demographics, clinical courses, endoscopic findings, and disease-specific therapies were obtained in adults >= 18 years with CVID-IBD over a 5-year period.Results We identified 11 patients with CVID-IBD, 6 with Crohn's disease (CD), and 5 with ulcerative colitis (UC). The presenting symptoms in 10 (91%) were abdominal pain and diarrhea. A majority of patients were diagnosed with IBD before CVID. Patients with CVID-IBD were diagnosed with CVID at an older age compared to patients with CVID only. The most prevalent endoscopic finding in CD was either erythema or erosions in the ileum, whereas in UC, it was pancolitis. One patient with CD and 1 with UC required treatment with immunomodulators to achieve remission. Three patients with CD and 2 with UC required treatment with biologics to achieve remission. Eight received intravenous immunoglobulin (IVIG) for CVID. Four patients with CD and 1 with UC were diagnosed with either leukemia or lymphoma, and the most common malignancy was chronic lymphocytic leukemia (n = 3).Conclusions This is one of few studies evaluating demographic, clinical, endoscopic, histologic features, and therapeutic management in patients with CVID-IBD. Further studies are required to elucidate long-term differences in the clinical course and treatment of patients with CVID-IBD. This is a study dedicated to characterizing patients with concomitant common variable immunodeficiency and inflammatory bowel disease that will better assist clinicians in managing this unique patient population.