Abstract
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.
Highlights
Adult onset autoimmune enteropathy (AIE) is a rare condition characterized by diarrhea refractory to dietary therapy diagnosed in patients with evidence of autoimmune conditions
AIE is most often characterized (1) IgG subclass anti-epithelial cell antibodies, (2) preferential small bowel involvement, and (3) CD3+ alphabeta T-cell receptor (TCR)+ infiltrates; there is insufficient evidence to conclude CD4+ T-cells are solely responsible in all cases of AIE
These enteropathies may be associated with primary immune deficiencies (PIDs) such as the immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, common variable immune deficiency and selective IgA deficiency, or may occur in patients with auto-immune phenomena in the absence of PIDs where it is termed auto-immune enteropathy (AIE) [1]
Summary
Our patient likely had an auto-immune enteropathy associated with an aberrant CD8+CD7- T-cell IEL. Consent Written informed consent was obtained and the study was approved by the institutional review board of the University of Kentucky Medical Center. Author details 1Department of Internal Medicine, University of Kentucky Medical Center, 800 Rose Street, Lexington, Kentucky, 40536, USA. Authors’ contributions SB analyzed the data and drafted the manuscript. VA, DV and RA provided human tissue samples and edited the manuscript (RA). EL analyzed and provided the histologic sections and flow cytometric data. WdW analyzed data and edited the manuscript. All authors have read and approved the final manuscript. Competing interests The authors declare that they have no competing interests
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