P232 Common variable immunodeficiency-associated enteropathy in consecutive patients from a tertiary hospital

Abstract

Abstract Background Common variable immunodeficiency (CVID) patients may develop intestinal inflammatory involvement in 9-20% of cases, although its diagnosis, management and prognosis are not well established. Methods We performed a transversal study with retrospective data collection. We reviewed all clinical records from consecutive adult CVID patients followed in Gregorio Marañón Hospital (Spain) from January 1st 1990 to January 1st 2023. A diagnosis of CVID-associated enteropathy (CVID-E) was established if patchy intestinal inflammation (Crohn-like pattern) or colonic inflammation (ulcerative colitis-like pattern) was demonstrated, or villous atrophy was observed in duodenal biopsies (celiac-like pattern) or inflammatory infiltrate was observed in colonic biopsies (microscopic colitis-like pattern). Differences between CVID-E patients and the rest of the CVID cohort were evaluated using chi-square and T student methods, when appropriate. Results 89 patients with confirmed CVID following ESID criteria were included. Patients’ characteristics are summarised in Table 1. All patients were receiving intravenous or subcutaneous Igs and reached an Ig G trough level >1000 mg/dL. CVID-E was diagnosed in 26 patients (29,2%), at a median of 6,4 years after CVID diagnosis. Surprisingly, 10 patients (38,5%) were diagnosed with CVID-E before or at the same time of CVID diagnosis. 5 patients were dead (19,2%), and the causes of death were gastric cancer (1 patient), liver complications (1), infectious complications (1), enteropathy (1) and unknown cause (1). 73,1% suffered from GI infections (Giardia lamblia, Campylobacter jejuni and Clostridioides difficile were the most frequent agents). The most frequent pattern of the disease was Crohn-like disease (Figure 1). Although exclusive celiac-like pattern was found in only 3 patients, 5 Crohn-like, 4 UC-like and 2 microscopic colitis-like pattern patients also presented duodenal atrophy. Unexpectedly, 46,2% of CVID-E patients suffered from concomitant liver involvement. Eight patients received corticosteroids, 2 azathioprine and 1 patient received infliximab and subsequent ustekinumab. One patient needed subtotal colectomy, another ileal resection and another patient received a terminal colostomy due to severe perianal disease. CVID-E patients presented statistically more frequent liver involvement, more GI infections, and a tendency towards more GI cancer. Conclusion CVID-E is frequent among CVID patients. These patients suffer from more GI infections, liver involvement and probably more GI cancer. Screening of levels of Igs at diagnosis of IBD and celiac disease is advisable as CVID diagnosis can occur at the same time or even after enteropathy diagnosis.