Journal of Clinical Immunology

, Volume 28, Supplement 1, pp 56–61 | Cite as

Autoimmunity in IgA Deficiency: Revisiting the Role of IgA as a Silent Housekeeper

  • Cristina M. A. Jacob
  • Antonio C. Pastorino
  • Kristine Fahl
  • Magda Carneiro-Sampaio
  • Renato C. Monteiro


Both systemic and organ-specific autoimmune diseases are major manifestations of IgA deficiency (IgAD), the most common primary immunodeficiency. In addition, to discuss the clinical findings of IgAD patients, we proposed a hypothesis to explain the high association with autoimmune phenomena. Based on observations, interactions of monomeric IgA with FcαRI result in a partial phosphorylation of FcRγ-associated FcαRI, notably in the immunoreceptor tyrosine-based activation motif (ITAM) inducing the recruitment of the SHP-1 tyrosine phosphatase. This leads to deactivation of several activating pathways of the immune system including immunoreceptors that bear ITAM motif and ITAM-independent receptors. Consequently, inflammatory reactions and auto-immune process would be prevented.


IgA deficiency autoimmunity clinical evolution Fc receptor physiopathology 



This work is supported by Fundação de Amparo à Pesquisa do Estado de São Paulo-FAPESP-grant 2002/05880-4.


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Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Cristina M. A. Jacob
    • 1
  • Antonio C. Pastorino
    • 1
  • Kristine Fahl
    • 1
  • Magda Carneiro-Sampaio
    • 1
  • Renato C. Monteiro
    • 2
    • 3
  1. 1.Instituto da Criança, Hospital das Clinicas da Faculdade de MedicinaUniversidade de São PauloSão PauloBrasil
  2. 2.Institut National de la Santé et de la Recherche Médicale U699ParisFrance
  3. 3.Université Paris 7, Faculté de Médicine Denis Diderot, Hôpital BichatParisFrance

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