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P-001 YI Inflammatory Bowel Disease and Common Variable Immunodeficiency—A Case Series

BACKGROUND:Common variable immunodeficiency (CVID) is the most clinically relevant primary immunodeficiency, and it may affect the entire gastrointestinal tract. CVID colitis findings include cryptitis and granulomas, mimicking inflammatory bowel disease (IBD), and CVID colitis may respond to treatments used for either Crohn’s disease (CD) or ulcerative colitis (UC). Furthermore, IBD and CVID may occur concurrently in the same patient. We sought to identify patients with both CVID and IBD and to describe clinical features and outcomes. METHODS:We used a centralized electronic database to identify potential cases of CVID and either CD or UC evaluated at our institution between January 1, 2000 and June 30, 2014. Medical records were reviewed to obtain the following information: location and behavior of IBD, medication use including anti-TNF agents and immunomodulators, IBD-related hospitalizations and surgeries, date of CVID diagnosis, and need for treatment with intravenous immunoglobulin. RESULTS:The cohort included 14 patients—10 patients with CVID and CD (CVID + CD), 2 patients with CVID and UC (CVID + UC), and 2 patients with CVID-enteropathy. Eight patients were females, and the median age for all patients at IBD diagnosis was 27.5 years (range, 9–47) and at CVID diagnosis was 25.5 years (0.5–80). Seven of the 12 patients with IBD were diagnosed with CVID after their diagnosis of IBD; specifically, all 7 of these patients had a diagnosis of CD. Five of these patients had persistent sinusitis or other respiratory infections that prompted evaluation of immunoglobulin levels and eventually a diagnosis of CVID. Of the 5 patients who were diagnosed with IBD after CVID, 4 patients initially presented with blood per rectum while the other patient presented with pure diarrhea. All patients were treated with intravenous immunoglobulin. The behavior of CD in the majority of these patients (n = 7) was classified as B1 based on the Montreal classification, and the location of CD had a wide distribution (3 ileal, 5 ileocolonic, and 2 colonic). Eight patients were exposed to anti-TNF agents (7 CD + CVID, 1 CVID-enteropathy), 6 patients had used immunomodulators (all CVID + CD), and 8 patients were exposed to corticosteroids (6 CVID + CD, 1 CVID-enteropathy, 1 CVID + UC). A total of 3 patients were hospitalized (2 CVID+ CD, 1 CVID + UC) and 6 patients underwent surgery (5 CVID + CD, 1 CVID + UC). CONCLUSIONS:Most patients in our cohort with overlapping CVID and IBD had CD rather than UC. Those with CVID + CD were more frequently exposed to anti-TNFs, immunomodulators, and corticosteroids and more frequently hospitalized compared to those with CVID + UC or CVID-enteropathy. The majority of patients with CVID + CD were diagnosed with CVID after CD diagnosis. Clinicians should be aware of CVID in those with IBD, and especially in those with CD as these patients may require closer follow-up to prevent complications. Finally, clinicians should maintain a high level of suspicion for CVID in a patient with IBD and chronic respiratory infections or sinusitis.