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Journal of Clinical Immunology

Erschienen in:

01.05.2008

Primary Immune Deficiency Disorders Presenting as Autoimmune Diseases: IPEX and APECED

verfasst von: D. Moraes-Vasconcelos, B. T. Costa-Carvalho, T. R. Torgerson, H. D. Ochs

Erschienen in: Journal of Clinical Immunology | Sonderheft 1/2008

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Abstract

Background

Several primary immune deficiency disorders are associated with autoimmunity and malignancy, suggesting a state of immune dysregulation. The concept of immune dysregulation as a direct cause of autoimmunity in primary immune deficiency disorders (PIDDs) has been strengthened by the recent discovery of distinct clinical entities linked to single-gene defects resulting in multiple autoimmune phenomena including immune dysregulation, polyendocrinopathy, enteropathy and X-linked (IPEX) syndrome, and autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy (APECED) syndrome.

Conclusion

Reviewing recent advances in our understanding of the small subgroup of PIDD patients with defined causes for autoimmunity may lead to the development of more effective treatment strategies for idiopathic human autoimmune diseases.

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Titel: Primary Immune Deficiency Disorders Presenting as Autoimmune Diseases: IPEX and APECED
verfasst von: D. Moraes-Vasconcelos
B. T. Costa-Carvalho
T. R. Torgerson
H. D. Ochs
Publikationsdatum: 01.05.2008
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe Sonderheft 1/2008
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-008-9176-5

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