Adult Autoimmune Enteropathy: Mayo Clinic Rochester Experience

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Adult Autoimmune Enteropathy: Mayo Clinic Rochester Experience Salma Akram, Joseph A. Murray, Darrell S. Pardi, Glenn L. Alexander, John A. Schaffner, Pierre A. Russo, Susan C. Abraham  Clinical Gastroenterology and Hepatology  Volume 5, Issue 11, Pages 1282-1290 (November 2007) DOI: 10.1016/j.cgh.2007.05.013 Copyright © 2007 AGA Institute Terms and Conditions

Figure 1 Anti-enterocyte antibodies. Indirect immunofluorescence with the patient’s serum on frozen section of normal human small bowel shows a linear fluorescence pattern along the brush border of the enterocytes (original magnification, 300×). Clinical Gastroenterology and Hepatology 2007 5, 1282-1290DOI: (10.1016/j.cgh.2007.05.013) Copyright © 2007 AGA Institute Terms and Conditions

Figure 2 Anti-goblet antibodies. Immunofluorescence staining in the goblet cells mucus, sparing the enterocytes (original magnification, 100×). Clinical Gastroenterology and Hepatology 2007 5, 1282-1290DOI: (10.1016/j.cgh.2007.05.013) Copyright © 2007 AGA Institute Terms and Conditions

Figure 3 Histopathologic findings in a patient with autoimmune enteritis associated with AG antibodies. (A) Low-power view of the jejunum shows near-total villous atrophy and dense lymphoplasmacellular inflammation in the lamina propria. (B) At higher power, there is surface epithelial injury with disruption of the brush border (arrow) and intense neutrophilic inflammation including crypt abscesses (asterisk). There is no abnormal lymphocytosis in the surface epithelium. Clinical Gastroenterology and Hepatology 2007 5, 1282-1290DOI: (10.1016/j.cgh.2007.05.013) Copyright © 2007 AGA Institute Terms and Conditions

Figure 4 Histopathologic findings in a patient with AG antibodies and pan-gastrointestinal autoimmune disease. (A) Duodenal biopsy demonstrates moderate villous blunting and absence of goblet cells; there is no abnormal surface lymphocytosis. (B) Colonic biopsy with absent goblet cells, mild lymphocytosis in the deep crypt epithelium (arrow), and crypt apoptosis (arrowhead). (C) Gastric biopsy taken from the fundus demonstrates features of autoimmune atrophic gastritis, with complete absence of oxyntic glands and partial replacement by pseudopyloric metaplasia (arrow). Clinical Gastroenterology and Hepatology 2007 5, 1282-1290DOI: (10.1016/j.cgh.2007.05.013) Copyright © 2007 AGA Institute Terms and Conditions

Figure 5 Histopathologic features in overlap between AIE and CD. (A) The duodenal biopsy shows near-total villous blunting with crypt architectural distortion including crypt branching (arrow) and crypt destruction (arrowhead), both unusual features of “pure” CD. (B) At high-power, there is intense surface intraepithelial lymphocytosis typical for CD, but there is also neutrophilic cryptitis (arrow) and scattered neutrophils in the surface epithelium. This patient had positive AE antibodies, HLA type DQ2, and a weakly positive IgA tTG antibody. Clinical Gastroenterology and Hepatology 2007 5, 1282-1290DOI: (10.1016/j.cgh.2007.05.013) Copyright © 2007 AGA Institute Terms and Conditions