title: The enteropathy associated with common variable immunodeficiency: the delineated frontiers with celiac disease.
abstract: 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.
authors.affiliation: Université Paris Descartes, France. georgia.malamut@egp.aphp.fr
authors.forename: Georgia
authors.initials: G
authors.lastname: Malamut
authors.forename: Virginie
authors.initials: V
authors.lastname: Verkarre
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Lascaux
authors.forename: Jacques
authors.initials: J
authors.lastname: Cosnes
authors.forename: Yoram
authors.initials: Y
authors.lastname: Bouhnik
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.forename: Dominique
authors.initials: D
authors.lastname: Béchade
authors.forename: Marianne
authors.initials: M
authors.lastname: Ziol
authors.forename: Anne
authors.initials: A
authors.lastname: Lavergne
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Nadine
authors.initials: N
authors.lastname: Cerf-Bensussan
authors.forename: Christophe
authors.initials: C
authors.lastname: Cellier
mesh_terms: 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