Innate and adaptive immunity in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

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.