Common variable immunodeficiency (CVID) is a rare primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections especially in respiratory and gastrointestinal systems. The authors present here a case of CVID with uncommon gastrointestinal manifestation in the form of CVID enteropathy. Based upon the clinical presentation and histopatholic findings the patient was diagnosed as CVID enteropathy and treated with intravenous antibiotics and 3-4 wkly intravenous immunoglobulin infusion and he had responded poorly to gastrointestinal symptoms but the frequency of respiratory and skin infection reduced. CVID has heterogenous gastrointestinal manifestations; among them the CVID enteropathy is an uncommon presentation which responds poorly to the mainstay therapy of intravenous immunoglobulin infusion and represents a diagnostic as well as therapeutic challenge for treating physician.
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http://dx.doi.org/10.1007/s12098-011-0635-2 | DOI Listing |
J Clin Med
January 2025
Department of Pathology, Ghent University Hospital, Ghent University, 9000 Ghent, Belgium.
: Gastrointestinal diseases are a major cause of morbidity in common variable immunodeficiency disorder (CVID), clinically often mimicking other conditions including celiac disease and inflammatory bowel disease (IBD). Hence, diagnosis of CVID remains challenging. This study aims to raise awareness and highlight histopathological clues for CVID in intestinal biopsies, emphasizing diagnostic pitfalls for the pathologist/gastroenterologist.
View Article and Find Full Text PDFDig Dis Sci
January 2025
Department of Internal Medicine, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Medicine, Chicago, IL, USA.
Immunol Res
December 2024
Department of Immunology, North Bristol NHS Trust, Bristol, UK.
CVID (common variable immunodeficiency) is associated with a variety of gastrointestinal disorders including those mimicking Crohn's disease and ulcerative colitis. At present there is no clear trial data for the treatment of CVID enteropathy. There are no specific recommendations for treatment; however, it is commonly treated in a similar manner to inflammatory bowel disease, with corticosteroids, 5-aminosalicylates (5-ASA), azathioprine and anti-TNF therapy all being used.
View Article and Find Full Text PDFCurr Opin Allergy Clin Immunol
December 2024
University Medical Center Utrecht, Department of Clinical Immunology and Rheumatology.
Purpose Of Review: Common variable immunodeficiency enteropathy (CVID-E) is a noninfectious complication of CVID caused by chronic inflammation of the gastrointestinal (GI) tract. Based on literature, a paucity or lack of plasma cells, although not obligatory for diagnosis, is a pathognomonic feature of CVID and more frequent in CVID-E. However, there is no consensus on standardized histopathological analysis of this feature in biopsies.
View Article and Find Full Text PDFmSphere
November 2024
Department of Molecular Virology and Microbiology, Baylor College of Medicine (BCM), Houston, Texas, USA.
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