CVID and Enteropathy: A retrospective study

Abstract

Introduction

Common variable immune deficiency (CVID) is associated with non-infectious gastrointestinal disease. We aim to further characterize this group of patients and to ascertain the effect of immunosuppressive treatments on the clinical course.

Method

We identified patients with enteropathy in two Dutch academic teaching hospitals and approached them during outpatient clinic visits. Using the electronic patient records, we retrospectively collected information in the national PID database until 2023.

Results

We included 39 patients with CVID and enteropathy (CVID-E). Bronchiectasis (69,2%), lymphoproliferation (46,1%), splenomegaly (35,9%) and autoimmunity (33,3%) were the most frequent co-occurring symptoms in CVID-E. The most prevalent pathogens included Giardia lamblia (23,1%), Campylobacter jejuni (17,9%). The most common endoscopy findings consisted of Inflammation (72,2%), erythema (69,4%) and NLH (50,0%). The most common histopathologic findings consisted of IBD-like inflammation (55,6%), indiscriminate chronic inflammation (47,2%), indiscriminate active inflammation (38,9%), IEL (36,1%) and villous atrophy (27,8%). Of all 15 complete remissions observed seven were with either monotherapy azathioprine or combination therapy including azathioprine (total of five patients). Four complete remissions were observed with either TNFi monotherapy or combination therapy with TNFi.

Conclusion

In summary, we found azathioprine and TNFi as monotherapy as well in combination therapy to have the best results in this cohort. Furthermore, CVID-E patients often suffer from inflammatory and infectious complications. Lastly, where in other distinct diseases one histopathological finding may dominate, in our patients many findings co-occurred.