AB1468 ENTEROPATHY IN DUTCH COMMON VARIABLE IMMUNODEFICIENCY COHORT

Background Common variable immunodeficiency (CVID) is the most common severe primary antibody deficiency. Although infections are prevalent clinical manifestations, a large amount of patients also suffer from immune dysregulation. This results in significant morbidity [1]. Different manifestations enteropathy can occur, yet little information available regarding its optimal treatment. Objectives Here we describe characteristics, endoscopy, histopathology and treatment response CVID (CVID-E) Dutch teaching hospital. Methods We included our hospital, that provided written informed consent. collected, retrospectively, gender, age start symptoms, at diagnosis. Reports endoscopies histopathology, course related symptoms was recorded for each patient alongside immunosuppressive medication (IS) used over time. Treatment defined as remission, response, need step-up, unknown. Descriptive statistics were to study associations. Results 32 82 UMC Utrecht had enteropathy. The average this group 45.4 (13.5 standard deviation, SD) mean diagnosis 31.7 (16.7 SD). first presentation 30.9 years (14.6 16 female. 28 (90.6%) CVID-E other complications: inflammatory, infectious malignant. 26 (81.3%) inflammatory manifestations; lymphoproliferation (43.8%) splenomegaly (34.4%) autoimmune disease (37.5%). Infectious complication bronchiectasis prevalence 68.8% cohort. Two solid malignancy one lymphoma. compared treated with IS those who not IS. Autoimmune cytopenia, significantly more IS, than without IS: 23.5% vs 0% (P<0.05) 82.4% 46.7% (p<0.01) respectively. 30 underwent 29 coloscopy 25 (78.1%) gastroscopy. histopathologic findings consisted bowel disease-like colitis (n=10) intra-epithelial lymphocytosis (n=9). Other note lymphocytic colitis(n=3), microscopic (n=2), collagenic (n=1) apoptotic enteritis (n=1). 17 (53.1%) received CVID-E. 14 local corticosteroids, 12 systemic 15 DMARDs (methotrexate, azathioprine, mesalazine, baricitinib, mycophenolate, tacrolimus sulfasalazine), 6 biologicals (adalimumab, infliximab ustekinumab) combined therapy. These 64 treatments. Local CS therapy did result remission. 9/17 (52.9%) needed 2 drugs. DMARD monotherapy achieved remission or 13 (46.2%). best concerning cohort seemed consist combinations DMARD(s) CS, TNF-alpha inhibitors (TNFi). Conclusion strongly associated complications bronchiectasis, especially on consists largest series reporting efficacy found combination be effective Additionally, TNFi seem have good enteropathy, when References [1]Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity mortality deficiency 4 decades. Blood 2012; 119:1650–1658. [2]Tangye SG, Al-Herz W, Bousfiha A, C, Franco JL, Holland SM, Klein Morio T, Oksenhendler E, Picard Puel A. Human inborn errors immunity: 2022 update classification international union immunological societies expert committee. J Clin Immunol 2022; 42:1473-507. Acknowledgements: NIL. Disclosure Interests None Declared.