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Flow Cytometry Study of Blood Cell Subtypes Reflects Autoimmune and Inflammatory Processes in Autoimmune Polyendocrine Syndrome Type I
Author(s) -
Wolff A. S. B.,
Oftedal B. E. V.,
Kisand K.,
Ersvær E.,
Lima K.,
Husebye E. S.
Publication year - 2010
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.2010.02397.x
Subject(s) - autoimmune regulator , autoimmunity , chronic mucocutaneous candidiasis , immunology , immunophenotyping , flow cytometry , immune system , autoimmune disease , medicine , cxcr3 , immune dysregulation , foxp3 , biology , antibody , disease , chemokine , chemokine receptor
Autoimmune polyendocrine syndrome type I (APS I) is a recessive disorder caused by mutations in the autoimmune regulator ( AIRE ) gene. AIRE is expressed in medullary epithelial cells where it activates transcription of organ‐specific proteins in thymus, thereby regulating autoimmunity. Patients with APS I have, in addition to autoimmune manifestations in endocrine organs, also often ectodermal dystrophies and chronic mucocutaneous candidiasis. The aim of this study was to characterize immune cell subpopulations in patients with APS I and their close relatives. Extensive blood mononuclear cell immunophenotyping was carried out on 19 patients with APS I, 18 first grade relatives and corresponding sex‐ and age‐matched healthy controls using flow cytometry. We found a significant relative reduction in T helper cells coexpressing CCR6 and CXCR3 in patients with APS I compared to controls (mean = 4.10% versus 5.94% respectively, P = 0.035). The pools of CD16 + monocytes and regulatory T cells (Tregs) were also lower in patients compared with healthy individuals (mean = 15.75% versus 26.78%, P = 0.028 and mean = 4.12% versus 6.73%, P = 0.029, respectively). This is the first report describing reduced numbers of CCR6 + CXCR3 + T helper cells and CD16 + monocytes in patients with APS I We further confirm previous findings of reduced numbers of Tregs in these patients.