Abstract
The basic derangement leading to immune destruction of endocrine glands in APECED is unknown. We studied lymphocyte subsets in 42 patients yith APECED and in 21 healthy controls. Lymphocytes were treated with monoclonal antibodies detecting pan-T (OKT3), T-helper (OKT4), T-suppressor/killer (OKT8), E-rosetting (OKT11) T-lymphocytes, and surface immunoglobulin (SIG)-positive B-lymphocytes, and analyzed by fluorescence activated cell sorter (FACS IV). The patients had higher lymphocyte counts than controls (2.71+1.20 vs 2.10+0.52 × 109/l; mean+SD; p=0.006). For all T-lymphocyte subsets studied, the dispersion of counts was wider for the patients than the controls. The mean counts were significantly different only for SIG-positive B-lymphocytes (0.50+0.28 vs 0.28+0.11 × 109/l; p=0. 0001) .The OKT4-OKT8-ratio was subnormal (>1.0) in 5 and supranormal (<3.1) in 5 patients. The deviations observed did not correlate with any indicator of the severity of APECED or presence of any component of it. Presence of circulating ovarian, testicular or placental antibodies in contrast to adrenal antibodies was associated with supranormal counts of SIG-positive cells (p=0.031, p=0.02, p=0.004, p=0.35; respectively). Presence of circulating thyroid microsomal or thyroglobulin antibodies, or parietal cell or islet cell antibodies did not correlate with any lymphocyte subset pattern. No single feature was characteristic of APECED. Presumably the lymphocyte subset deviations are secondary manifestations of APECED.
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Ahonen, P., Willebrand, E. & Perheentupa, J. LYMPHOCYTE SUBSETS AND AUTOANTIBODIES IN PATIENTS WITH AUTOIMMUNE POLYENDOCRINOPATHY - CANDIDOSIS ECTODERMAL DYSTROPHY (APECED). Pediatr Res 20, 1204 (1986). https://doi.org/10.1203/00006450-198611000-00183
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DOI: https://doi.org/10.1203/00006450-198611000-00183