2015
DOI: 10.1016/j.alit.2014.09.003
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Primary sclerosing cholangitis in common variable immune deficiency

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Cited by 13 publications
(25 citation statements)
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“…1 GI conditions manifest in 20 to 50% of CVID patients and include esophageal candidiasis, diarrhea, nodular lymphoid hyperplasia, autoimmune enteropathy, irritable bowel disease-like colitis, pernicious anemia, gastric adenocarcinoma, B cell immunophenotype lymphoma, and cholangitis. [2][3][4][5] Three studies have reported associations of cholangitis and CVID or primary immunodeficiences, [3][4][5] although we report the first case of recurrent ascending cholangitis as the primary manifestation of CVID. In addition, this case demonstrated the first reported successful treatment of this condition with oral human intravenous immunoglobulin (IVIG).…”
Section: Introductionmentioning
confidence: 85%
See 1 more Smart Citation
“…1 GI conditions manifest in 20 to 50% of CVID patients and include esophageal candidiasis, diarrhea, nodular lymphoid hyperplasia, autoimmune enteropathy, irritable bowel disease-like colitis, pernicious anemia, gastric adenocarcinoma, B cell immunophenotype lymphoma, and cholangitis. [2][3][4][5] Three studies have reported associations of cholangitis and CVID or primary immunodeficiences, [3][4][5] although we report the first case of recurrent ascending cholangitis as the primary manifestation of CVID. In addition, this case demonstrated the first reported successful treatment of this condition with oral human intravenous immunoglobulin (IVIG).…”
Section: Introductionmentioning
confidence: 85%
“…2 GI conditions are reported in up to 50% of CVID patients, with infectious diarrhea as the most common symptom. 1,3 Gastric adenocarcinoma, immune-mediated enteropathy, nodular lymphoid hyperplasia of the GI tract, small intestine bacterial overgrowth, small bowel villous atrophy, and gastritis have also been noted in CVID cases. 1 Hepatic manifestations, most commonly hepatitis and liver granulomas, are less frequent but have been documented in 9 to 12% of patients with CVID.…”
Section: Discussionmentioning
confidence: 99%
“…Cholangiographic evidence is diagnostic and liver biopsy is usually not needed . Primary sclerosing cholangitis has been reported in association with acquired immunodeficiency syndrome, CD40 ligand deficiency, and common variable immunodeficiency . Kahn et al.…”
Section: Discussionmentioning
confidence: 99%
“…13 Primary sclerosing cholangitis has been reported in association with acquired immunodeficiency syndrome, CD40 ligand deficiency, and common variable immunodeficiency. 14,15 Kahn et al 16 reported two children with classical WAS who developed primary sclerosing cholangitis at the ages of 14 and 5.5 years, respectively. One of them had an association with ulcerative colitis and hence sclerosing cholangitis was inferred to be autoimmune in nature.…”
Section: Discussionmentioning
confidence: 99%
“…Ad (4): Die sekundäre SC (SSC) unterscheidet sich von der PSC dadurch, dass diese oft im Rahmen der Langerhanszell-Histiozytose und anderer Immundefekte auftritt, in der Literatur aber oft der PSC zugeordnet wird [627,628]. Eine SSC wird auch bei der zystischen Fibrose, bei Psoriasis, Sichelzellanämie, Stammzelloder Lebertransplantation beobachtet [619].…”
Section: Kommentarunclassified