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Th17 Inflammation Model of Oropharyngeal Candidiasis in Immunodeficient Mice
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Innate and adaptive immunity in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.

R Perniola1, M Congedo, A Rizzo

  • 1Paediatric Unit, V. Fazzi Regional Hospital, Lecce, Italy. rperniola@hotmail.com

Mycoses
|April 11, 2008
PubMed
Summary

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) patients exhibit effective immune responses to pathogens. Despite chronic candidiasis, their phagocyte and lymphocyte functions are normal, suggesting unknown mechanisms for mucocutaneous anergy.

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Area of Science:

  • Immunology
  • Endocrinology
  • Genetics

Background:

  • Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive syndrome.
  • APECED is characterized by chronic mucocutaneous candidiasis (CMC) and endocrine failures.
  • Immune responses to infectious agents in APECED are not fully understood.

Purpose of the Study:

  • To investigate the efficiency of innate and adaptive immune responses in APECED patients.
  • To compare immune cell function and immunoglobulin levels between APECED patients and healthy controls.
  • To explore correlations between oral candidiasis and anti-Candida antibody titers.

Main Methods:

  • Flow cytometry was used to assess monocyte and polymorphonuclear leucocyte phagocytosis and bacterial/fungal killing.
  • Lymphocyte activation was measured in vitro using mitogens and Candida albicans.
  • Gamma-globulin and immunoglobulin concentrations were compared; correlations with oral fungal load were analyzed.

Main Results:

  • APECED patients showed normal phagocyte function and lymphocyte activation, despite a higher monocyte count.
  • Elevated gamma-globulin levels were observed in APECED patients.
  • Anti-Candida IgM and IgG correlated with current and past oral candidiasis, respectively.

Conclusions:

  • APECED patients possess efficient innate and adaptive immune responses against exogenous stimuli.
  • The mechanisms underlying mucocutaneous anergy and high prevalence of CMC in APECED remain unclear.
  • Further research is needed to elucidate the specific pathways leading to CMC in this syndrome.