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Allergologia et Immunopathologia
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Inicio Allergologia et Immunopathologia From autoimmune enteropathy to the IPEX (immune dysfunction, polyendocrinopathy,...
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Vol. 37. Num. 4.July - August 2009
Pages 173-222
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Vol. 37. Num. 4.July - August 2009
Pages 173-222
DOI: 10.1016/j.aller.2009.04.002
From autoimmune enteropathy to the IPEX (immune dysfunction, polyendocrinopathy, enteropathy, X-linked) syndrome
A. Blanco Quirósa,??
Corresponding author

Corresponding author.
, E. Arranz Sanza, D. Bernardo Ordiza, J.A. Garrote Adradosa,b
a Department of Pediatrics and Immunology, IBGM, University of Valladolid, Spain
b Clinical Research Unity, Hospital Clínico Universitario, Valladolid, Spain
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Figures (4)
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Tables (4)
Table 1. Different forms of intractable diarrhoea of infancy
Table 2. Ancient terms assigned to IPEX syndrome (Marabelle et al. 2008)
Table 3. Differential characteristics of X-linked syndromes with immune abnormalities (Bennett et al. 2001)
Table 4. Simplified view of protective role and immunopathology involvement of different CD4+ T-cell subset (Romagnani 2006)
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The term autoimmune enteropathy (AIE) was applied to a form of “intractable diarrhoea” with serum gut autoantibodies, characterized by male predominance, early onset, poor response to parenteral nutrition and several autoimmune diseases, mainly type 1 diabetes. In recent years the vague concept of AIE has became more precise thanks to the discovery of its genetic and molecular basis. The FOXP3 molecule is crucial for the generation and maturation of regulatory T cells (Treg) expressing CD4+ and CD25+ molecules. Mutations of the FOXP3 gene, located in X chromosome, produce a syndrome with Immune dysfunction, Polyendocrinopathy, Enteropathy and X-linked inheritance (IPEX). The majority of the ancient so-called AIE cases probably correspond to the new IPEX syndrome, even in female patients who may have some autosomal genetic variants. Besides FOXP3, other molecules are likely to be involved in the generation and function of Treg and its deficiency may also enhance autoimmune disease and IPEX-like syndromes. Meanwhile, the important pathogenic role previously ascribed to gut autoantibodies has vanished, with it remaining as having only certain screening usefulness.

Autoimmune enteropathy
IPEX syndrome
FOXP3 molecule
Regulatory T cells


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