title: Innate and adaptive immunity in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.
abstract: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive syndrome characterised by chronic mucocutaneous candidiasis (CMC) and multiple endocrine failures. While the spectrum and modalities of autoimmunity are the main objects of current research into APECED, unequivocal data on the efficiency of immune responses to infectious agents are still elusive. The in vitro ability of monocytes and polymorphonuclear leucocytes to phagocytise and kill bacteria and fungi, and the degree of activation of lymphocytes cultured with mitogens and Candida albicans were investigated by flow cytometry in 11 APECED patients and healthy subjects. In addition, a comparison of gamma-globulin and immunoglobulin (Ig) concentrations was performed, and a correlation was sought between oral fungal load and the anti-Candida antibody titre. No difference between APECED patients and healthy subjects was observed in the phagocyte function, although the patients had a larger number of monocytes. Similarly, cultured lymphocytes were equally activated in the two groups. The concentration of gamma-globulins was higher among APECED patients, and anti-Candida IgM and IgG correlated with current and past oral candidiasis respectively. APECED patients have efficient innate and adaptive immune responses against exogenous stimuli, and currently, the mechanisms of mucocutaneous anergy leading to the high prevalence of CMC in this syndrome remain to be elucidated.
authors.affiliation: Paediatric Unit, V. Fazzi Regional Hospital, Lecce, Italy. rperniola@hotmail.com
authors.forename: R
authors.initials: R
authors.lastname: Perniola
authors.forename: M
authors.initials: M
authors.lastname: Congedo
authors.forename: A
authors.initials: A
authors.lastname: Rizzo
authors.forename: A
authors.initials: A
authors.lastname: Sticchi Damiani
authors.forename: M L
authors.initials: ML
authors.lastname: Faneschi
authors.forename: M
authors.initials: M
authors.lastname: Pizzolante
authors.forename: G
authors.initials: G
authors.lastname: Lobreglio
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000032:analysis*; D015551:Autoimmunity; D002178:Candidiasis, Chronic Mucocutaneous / Q000276:immunology*; D005260:Female; D006801:Humans; D007113:Immunity, Innate; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D008297:Male; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications / Q000235:genetics / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D007074:Immunoglobulin G; D007075:Immunoglobulin M