OBJECTIVES: 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.
OBJECTIVES: 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 CVIDpatients 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: Andrea Cerutti; Montserrat Cols; Maurizio Gentile; Linda Cassis; Carolina M Barra; Bing He; Irene Puga; Kang Chen Journal: Ann N Y Acad Sci Date: 2011-11 Impact factor: 5.691
Authors: Francisco A Bonilla; Isil Barlan; Helen Chapel; Beatriz T Costa-Carvalho; Charlotte Cunningham-Rundles; M Teresa de la Morena; Francisco J Espinosa-Rosales; Lennart Hammarström; Shigeaki Nonoyama; Isabella Quinti; John M Routes; Mimi L K Tang; Klaus Warnatz Journal: J Allergy Clin Immunol Pract Date: 2015-11-07
Authors: Silje F Jørgensen; Henrik M Reims; Didrik Frydenlund; Kristian Holm; Vemund Paulsen; Annika E Michelsen; Kristin K Jørgensen; Liv T Osnes; Jorunn Bratlie; Tor J Eide; Christen P Dahl; Ellen Holter; Rune R Tronstad; Kurt Hanevik; Hans-Richard Brattbakk; Fatemeh Kaveh; Torunn Fiskerstrand; Anne-Marte B Kran; Thor Ueland; Tom H Karlsen; Pål Aukrust; Knut E A Lundin; Børre Fevang Journal: Am J Gastroenterol Date: 2016-08-16 Impact factor: 10.864
Authors: Ivan J Fuss; Julia Friend; Zhiqiong Yang; Jian Ping He; Lubna Hooda; James Boyer; Liqiang Xi; Mark Raffeld; David E Kleiner; Theo Heller; Warren Strober Journal: J Clin Immunol Date: 2013-02-19 Impact factor: 8.317