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- W2920973354 abstract "Common Variable Immunodeficiency (CVID) is a disorder of the humoral immune system. Gastrointestinal (GI) manifestations occur in 40-50% of cases, and presenting signs and symptoms can vary. We present a case of CVID with markedly decreased immunoglobulin levels and multiple GI pathologies. A 55-year-old male with history of lymphocytic colitis, but otherwise limited medical contact, presented for admission with neuropathy, chronic diarrhea, and had lost 40kg. His BMI was < 15. Laboratory data are outlined in Table 1, and are notable for non-anion gap acidosis, immunoglobulin deficiency, and vitamin/mineral deficiencies. These findings suggested a diagnosis of CVID. Stool testing was positive for Campylobacter and Norovirus. Endoscopy demonstrated gastritis, extensive intestinal metaplasia, and hypogammaglobulinemic sprue which did not respond to gluten avoidance. Colonic biopsies demonstrated rare to absent plasma cells, and areas of nodular lymphoid hyperplasia, consistent with his prior history of lymphocytic colitis. He responded robustly to treatment (intravenous immunoglobulin, enteric budesonide, levofloxacin, vitamin/mineral repletion) and avoided tube feeding as well as parenteral nutrition. GI manifestations of CVID include infection, inflammation, and malignancy. The responsible infectious agents in CVID are multiple, but Giardia, Campylobacter, Salmonella, and Norovirus are disproportionately common. Non-infectious enteropathy, similar to celiac disease or inflammatory bowel disease (IBD), may lead to significant malabsorption and may require parenteral nutrition. Interestingly, celiac-like villous atrophy (or hypogammaglobulinemic sprue) rarely responds to gluten avoidance and clearance of Norovirus is associated with histological improvement. Diagnosis of GI malignancy in CVID is notoriously challenging. Non-Hodgkin's lymphoma and gastric cancer is especially prevalent, but diagnoses may be erroneous due to the fact that organomegaly and GI lymphoid hyperplasia (sometimes with monoclonality) is a benign coincidence. Some authors have recommended CVID-specific surveillance algorithms for gastric cancer based on endoscopic findings of gastritis, metaplasia, or dysplasia. The varying range and frequency of gastrointestinal manifestations of CVID place gastroenterologists in a challenging position to diagnose and comprehensively care for patients.1590 Figure 1. Laboratory Data" @default.
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- W2920973354 date "2018-10-01" @default.
- W2920973354 modified "2023-09-27" @default.
- W2920973354 title "A Patient Near Death - And Common Variable Immune Deficiency to Blame" @default.
- W2920973354 doi "https://doi.org/10.14309/00000434-201810001-01590" @default.
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