Autoimmune enteropathy with a CD8+ CD7-T-cell small bowel intraepithelial lymphocytosis: case report and literature review

BMC Gastroenterology

Case Report

Shrinivas Bishu¹  Eun Y Lee²  H David Vargas³  Razvan Arsenescu⁴  Willem JS de Villiers⁴  Violeta Arsenescu⁴ 

[1] Department of Internal Medicine, University of Kentucky Medical Center, 800 Rose Street, 40536, Lexington, Kentucky, USA;Department of Pathology and Laboratory Medicine, University of Kentucky Medical Center, 800 Rose Street, 40536, Lexington, Kentucky, USA;Department of Surgery, University of Kentucky Medical Center, 800 Rose Street, 40536, Lexington, Kentucky, USA;Division of Digestive Diseases and Nutrition, University of Kentucky Medical Center, 800 Rose Street, 40536, Lexington, Kentucky, USA;

关键词: Celiac Disease;    Villous Atrophy;    Common Variable Immune Deficiency;    Tropical Sprue;    Refractory Celiac Disease;   

DOI  :  10.1186/1471-230X-11-131

 received in 2011-03-11, accepted in 2011-11-29,  发布年份 2011

来源: Springer

【 摘 要 】

BackgroundAdult 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.Case PresentationWe 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.ConclusionsAIE 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.

【 授权许可】

CC BY   
© Bishu et al; licensee BioMed Central Ltd. 2011