title: Autoimmune enteropathy with a CD8+ CD7- T-cell small bowel intraepithelial lymphocytosis: case report and literature review.
abstract: BACKGROUND
  Adult onset autoimmune enteropathy (AIE) is a rare condition characterized by diarrhea refractory to dietary therapy diagnosed in patients with evidence of autoimmune conditions. Auto-antibodies to gut epithelial cells and other tissues are commonly demonstrated. Despite increasing awareness, the pathogenesis, histologic, immunologic and clinical features of AIE remain uncertain. There remains controversy regarding the diagnostic criteria, the frequency and types of auto-antibodies and associated autoimmune conditions, and the extent and types of histologic and immunologic abnormalities. CD4+ T-cells are thought to at least responsible for this condition; whether other cell types, including B- and other T-cell subsets are involved, are uncertain. We present a unique case of AIE associated with a CD8+CD7- lymphocytosis and review the literature to characterize the histologic and immunologic abnormalities, and the autoantibodies and autoimmune conditions associated with AIE.
  
  
  METHODS
  We present a case of immune mediated enteropathy distinguished by the CD8+CD7- intra-epithelial and lamina propria lymphocytosis. Twenty-nine cases of AIE have been reported. The majority of patients had auto-antibodies (typically anti-enterocyte), preferential small bowel involvement, and predominately CD3+ CD4+ infiltrates. Common therapies included steroids or immuno-suppressive agents and clinical response with associated with histologic improvement.
  
  
  CONCLUSIONS
  AIE is most often characterized (1) IgG subclass anti-epithelial cell antibodies, (2) preferential small bowel involvement, and (3) CD3+ alphabeta TCR+ infiltrates; there is insufficient evidence to conclude CD4+ T-cells are solely responsible in all cases of AIE.
authors: Department of Internal Medicine, University of Kentucky Medical Center, 800 Rose Street, Lexington, Kentucky 40536, USA. shrinivas@uky.edu
authors: Shrinivas
authors: S
authors: Bishu
authors: Violeta
authors: V
authors: Arsenescu
authors: Eun Y
authors: EY
authors: Lee
authors: H David
authors: HD
authors: Vargas
authors: Willem J S
authors: WJ
authors: de Villiers
authors: Razvan
authors: R
authors: Arsenescu
mesh_terms: D000328:Adult; D019011:Antigens, CD7 / Q000032:analysis; D016827:CD8 Antigens / Q000032:analysis; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000276:immunology* / Q000473:pathology; D007421:Intestine, Small / Q000276:immunology / Q000473:pathology; D008218:Lymphocytosis / Q000150:complications / Q000276:immunology*; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D019011:Antigens, CD7; D016827:CD8 Antigens