Abstract
Diarrhea is the commonest gastrointestinal symptom in patients with common variable immunodeficiency (CVID). Different pathologies in patients’ bowel biopsies have been described and links with infections have been demonstrated. The aim of this study was to analyze the bowel histology of CVID patients in the Royal-Free-Hospital (RFH) London CVID cohort. Ninety-five bowel histology samples from 44 adult CVID patients were reviewed and grouped by histological patterns. Reasons for endoscopy and possible causative infections were recorded. Lymphocyte phenotyping results were compared between patients with different histological features. There was no distinctive feature that occurred in most diarrhea patients. Out of 44 patients (95 biopsies), 38 lacked plasma cells. In 14 of 21 patients with nodular lymphoid hyperplasia (NLH), this was the only visible pathology. In two patients, an infection with Giardia lamblia was associated with NLH. An IBD-like picture was seen in two patients. A coeliac-like picture was found in six patients, four of these had norovirus. NLH as well as inflammation often occurred as single features. There was no difference in blood lymphocyte phenotyping results comparing groups of histological features. We suggest that bowel histology in CVID patients with abdominal symptoms falls into three major histological patterns: (i) a coeliac-like histology, (ii) IBD-like changes, and (iii) NLH. Most patients, but remarkably not all, lacked plasma cells. CVID patients with diarrhea may have an altered bowel histology due to poorly understood and likely diverse immune-mediated mechanisms, occasionally driven by infections.
Highlights
Common variable immunodeficiency (CVID) is the commonest symptomatic primary immunodeficiency in humans [1] with a wide spectrum of clinical complications [2]
Herbst et al found that a total absence of IgA1+2 and IgG2 producing plasma cells occurred very often in CVID patients and that a defect of intestinal immunoglobulin producing plasma cells correlates with lower levels of the respective isotype in the peripheral blood [10]
Duodenum the reason for endoscopy if available: D diarrhea, M malabsorption, A anemia, altBh altered bowel habits, (D) diarrhea was the reason for endoscopy, but it had settled at biopsy. i infection suspected, prot. protein losing enteropathy suspected, c coeliac disease suspected, dysp. dyspepsia, P abdominal pain, FOB fecal occult blood, wl weight loss, v vomiting
Summary
Common variable immunodeficiency (CVID) is the commonest symptomatic primary immunodeficiency in humans [1] with a wide spectrum of clinical complications [2]. Patients have very low immunoglobulin levels of IgG and IgA or IgM with a poor vaccination response due to impaired B-cell function, a predisposition to infection, and autoimmune or granulomatous manifestations [3]. Up to 47% of investigated CVID patients suffer from abdominal symptoms [4,5,6,7,8]. Studies on the histopathological correlates of CVIDrelated abdominal symptoms have shown a variety of findings in the small intestine. Herbst et al found that a total absence of IgA1+2 and IgG2 producing plasma cells occurred very often in CVID patients and that a defect of intestinal immunoglobulin producing plasma cells correlates with lower levels of the respective isotype in the peripheral blood [10]
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