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

Shrinivas Bishu, Violeta Arsenescu, Eun Y. Lee, H. David Vargas, Willem J S de Villiers, Razvan Arsenescu

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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.Case Presentation: 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.

Original languageEnglish (US)
Article number131
JournalBMC Gastroenterology
Volume11
DOIs
StatePublished - Nov 29 2011

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Lymphocytosis
T-Lymphocytes
Antibodies
Epithelial Cells
Enterocytes
T-Lymphocyte Subsets
Autoimmune enteropathy
Autoantibodies
Anti-Idiotypic Antibodies
Diarrhea
Mucous Membrane
Steroids
Therapeutics

All Science Journal Classification (ASJC) codes

  • Gastroenterology

Cite this

Bishu, Shrinivas ; Arsenescu, Violeta ; Lee, Eun Y. ; Vargas, H. David ; de Villiers, Willem J S ; Arsenescu, Razvan. / Autoimmune enteropathy with a CD8 + CD7 - T-cell small bowel intraepithelial lymphocytosis : Case report and literature review. In: BMC Gastroenterology. 2011 ; Vol. 11.
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Autoimmune enteropathy with a CD8 + CD7 - T-cell small bowel intraepithelial lymphocytosis : Case report and literature review. / Bishu, Shrinivas; Arsenescu, Violeta; Lee, Eun Y.; Vargas, H. David; de Villiers, Willem J S; Arsenescu, Razvan.

In: BMC Gastroenterology, Vol. 11, 131, 29.11.2011.

Research output: Contribution to journalArticle

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T1 - Autoimmune enteropathy with a CD8 + CD7 - T-cell small bowel intraepithelial lymphocytosis

T2 - Case report and literature review

AU - Bishu, Shrinivas

AU - Arsenescu, Violeta

AU - Lee, Eun Y.

AU - Vargas, H. David

AU - de Villiers, Willem J S

AU - Arsenescu, Razvan

PY - 2011/11/29

Y1 - 2011/11/29

N2 - 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.Case Presentation: 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.

AB - 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.Case Presentation: 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.

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