The enteropathy associated with common variable immunodeficiency: the delineated frontiers with celiac disease.
OBJECTIVE
The enteropathy associated with common variable immunodeficiency (CVID) is poorly characterized, and its possible relationships with well-defined causes of enteropathy, such as celiac sprue (CS), remain debated. We aimed to assess the clinical and histopathological features of the enteropathy associated with CVID.


METHODS
The medical files of 50 CVID patients with gastrointestinal symptoms were analyzed retrospectively. Histological, phenotypic, and molecular analysis of intestinal endoscopic specimens was centrally performed.


RESULTS
Chronic diarrhea was the most frequent gastrointestinal symptom (92%), and biological evidence of malabsorption was observed in 54% of patients. Chronic gastritis associated or not with pernicious anemia and microscopic colitis were the most frequently observed histopathological features in gastric and colonic mucosa, respectively. Small-bowel biopsies available in 41 patients showed moderate increase in intestinal intraepithelial lymphocytes in 31 patients (75.6%) and villous atrophy in 21 patients (51%). Distinctive features from CS were a profound depletion in plasma cells and follicular lymphoid hyperplasia. Presence of peripheral blood CD8+ hyperlymphocytosis was predictive of intestinal intraepithelial hyperlymphocytosis. Intravenous (i.v.) immunoglobulin (Ig) therapy had no effect on enteropathy-related symptoms. Gluten-free diet improved only two out of 12 patients with villous atrophy, whereas all patients (7/7) responded to steroid therapy.


CONCLUSIONS
Several distinctive features differentiate CVID enteropathy from other causes of enteropathy including CS. Replacement i.v. Ig therapy is insufficient to improve gastrointestinal symptoms. Steroids are effective in reducing inflammation and restoring mucosal architecture.
Malamut
Georgia
G
Université Paris Descartes, France. georgia.malamut@egp.aphp.fr
Verkarre
Virginie
V
Suarez
Felipe
F
Viallard
Jean-François
JF
Lascaux
Anne-Sophie
AS
Cosnes
Jacques
J
Bouhnik
Yoram
Y
Lambotte
Olivier
O
Béchade
Dominique
D
Ziol
Marianne
M
Lavergne
Anne
A
Hermine
Olivier
O
Cerf-Bensussan
Nadine
N
Cellier
Christophe
C
D001284:Atrophy / Q000473:pathology; D002446:Celiac Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D003106:Colon / Q000473:pathology* / Q000503:physiopathology; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000150:complications / Q000503:physiopathology; D005756:Gastritis / Q000150:complications / Q000503:physiopathology; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology* / Q000503:physiopathology; D007421:Intestine, Small / Q000473:pathology* / Q000503:physiopathology; D008499:Medical Records; D010950:Plasma Cells / Q000473:pathology; D012189:Retrospective Studies