P754 Inflammatory bowel disease-like disease in patients with primary immunodeficiencies with chronic digestive symptoms: Diagnostic yield of colonoscopy

Background Primary immunodeficiencies (PIDs) are a heterogeneous group of diseases characterised by genetic defects in the immune system. The respiratory and gastrointestinal tracts are the most affected. Digestive involvement may be due to infections, immune-mediation or malignancy. Immune-mediated involvement is expressed clinically and endoscopically like an inflammatory bowel disease (IBD-like disease). The objective of this study was to describe the endoscopic and histological findings in patients with PID and chronic digestive symptoms studied by colonoscopy with a final diagnosis of IBD-like disease. Methods Between 2004 and 2017 we included 30 patients with PID, chronic digestive manifestations and at least one colonoscopy with negative stool microbiological studies. Results Of the patients included, 19 (63%) were women. The median age was 62 years (IQR 49–74). Seven (23%) presented selective IgA deficiency, 11 (37%) common variable immunodeficiency (CVID) and 12 (40%) hypogammaglobulinemia. The digestive symptoms reported were chronic diarrhea (73%), abdominal pain (73%) and weight loss (36%). Ten patients (33.3%) had IBD associated, 5 had a pattern of ulcerative colitis (UC), 3 of Crohn’s disease (CD) and 2 of non-classifiable IBD (NCIBD). Diarrhoea was the most common manifestation in those with IBD-like disease (100%). The most frequent-related PID was CVID (54%). Of the 5 UC-like patients, 4 were extensive and 1 proctitis; the 3 CD-like patients presented ileocolic location. Of the 10 patients with IBD-like disease colonoscopy showed: oedema (40%), loss of vascular pattern (40%), erosions (30%), ulcers (40%), pseudopolyps (10%) and 50% did not present endoscopic findings at the time of the study. Histological features were chronic inflammation (100%), cryptitis-pericryptitis-microabscesses (60%) and granulomas (10%). Additionally, we found the presence of apoptotic cells (40%) and absence of plasmocytes in the lamina propria (40%). Upper endoscopy was performed concomitantly in 24 patients, in 17% we found at duodenum nodularity and scalloping. Duodenal biopsies were taken in 15 patients and the histological features were: increased intraepithelial lymphocytes (40%), crypt hyperplasia (20%), atrophic mucosa (6.6%). Conclusion One third of patients with PID and chronic digestive symptoms who performed a colonoscopy presented IBD-like disease. CVID was the most associated PID with IBD-like disease. Half of the patients had no colonoscopic findings, so taking random biopsies is suggested. Histological findings were typical of IBD, being the presence of apoptotic bodies and the absence of plasma cells distinctive of the associated PID.