#137 – CLINICAL, ENDOSCOPIC AND HISTOLOGIC FEATURES OF COMMON VARIABLE IMMUNODEFICIENCY

Volumen 34, SPED 2023, Año 2023
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PDF | https://doi.org/10.46613/congastro2023-137

This work is licensed under CC BY 4.0

Fluxa D1, Alahmad M2, Loeb L1, Squire J1, Ha C3, Pardi D4, Bi Y1, Kinnucan J1, Picco M1, Leighton J3, Farraye F1, Hashash J1

1Mayo Clinic Florida, Jacksonville, Estados Unidos (EEUU)
2Sheikh Shakhbout, Abudhabi, Estados Unidos (EEUU)
3Mayo Clinic Arizona, Arizona, Estados Unidos (EEUU)
4Mayo Clinic Rochester, Rochester, Estados Unidos (EEUU)

  1. Introduction

Common variable immunodeficiency (CVID) is a rare illness with estimated prevalence of 1/25,000 individuals. Studies describing endoscopic and histopathological findings, and therapeutic options are lacking.

  1. Objectives

To describe gastrointestinal (GI) symptoms, endo and histo findings, and real-world experience treatment of patients with CVID enteropathy (CVIDe).

  1. Methods

Retrospective review of patients ≥16 years of age diagnosed with primary CVID who underwent endo evaluation for GI symptoms at a major three site academic medical center. Patients were identified by searching our institution’s database. Demographics, GI symptoms, endo and histo findings, and treatments were abstracted. Descriptive statistics were used to analyze the results.

  1. Results

A total of 57 patients were included; 53% female, median age at CVID diagnosis was 32 years (range 4-66). A total of 147 procedures were included. The most common GI symptoms leading to endo evaluation included diarrhea (50%), abdominal pain (29%), nausea (23%), weight loss (17%), vomiting (14%), and less frequently GI bleeding, bloating, anorexia/early satiety, heartburn, dysphagia, iron and vitamin B12 deficiency. Endoscopic and histopathological findings are described in Table 1. Twelve patients were diagnosed with CVIDe and received treatment with budesonide (9), prednisone (1), mesalamine (1), vedolizumab (2), infliximab (2), ustekinumab (1), sirolimus (1), abatacept (1), cholestyramine (1) in addition to intravenous or subcutaneous immunoglobulin. One patient underwent bone marrow transplant for medically refractory disease.

  1. Conclusion

GI symptoms are frequent in patients with CVID. While endoscopic and histopathological findings may be normal, decreased plasma cells and apoptosis are characteristic of CVIDe. In our series, 21% of the patients were diagnosed with CVIDe, similar to 15-20% as described by the literature. Most patients were treated with budesonide and 25% of patients eventually required biologics. Despite the lack of randomized controlled trials, patients with CVIDe may benefit from treatment with immunomodulators/biologics to reduce morbidity and mortality.