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

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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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Acknowledgments

NIH grants HD043376-03 (TRT) and HD37091 and grants from the Jeffrey Modell Foundation (HDO); FAPESP (grants 99/07399-7 and 02/05880-4) and CNPq (Brazilian Council for Research and Development).

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Authors and Affiliations

  1. Laboratory of Medical Investigation in Dermatology and Immunodeficiencies (LIM/56) and Primary Immunodeficiency Outpatient Unit (ADEE-3003), University of São Paulo School of Medicine, São Paulo, Brazil

    D. Moraes-Vasconcelos

  2. Division of Allergy, Clinical Immunology and Rheumatology, Department of Pediatrics, Federal University of São Paulo-Escola Paulista de Medicina, São Paulo, Brazil

    B. T. Costa-Carvalho

  3. Department of Pediatrics, University of Washington School of Medicine and Children’s Hospital and Regional Medical Center, Seattle, WA, USA

    T. R. Torgerson & H. D. Ochs

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  1. D. Moraes-Vasconcelos
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  2. B. T. Costa-Carvalho
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  3. T. R. Torgerson
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  4. H. D. Ochs
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Correspondence to D. Moraes-Vasconcelos.

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Moraes-Vasconcelos, D., Costa-Carvalho, B.T., Torgerson, T.R. et al. Primary Immune Deficiency Disorders Presenting as Autoimmune Diseases: IPEX and APECED. J Clin Immunol 28 (Suppl 1), 11–19 (2008). https://doi.org/10.1007/s10875-008-9176-5

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