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II. Adrenal Cortex and Steroid 21-Hydroxylase Autoantibodies in Children with Organ-Specific Autoimmune Diseases: Markers of High Progression to Clinical Addison’s Disease

Chair of Clinical Immunology and Allergy (C.B., M.V., R.Z., B.P., F.P.), and Chair of Endocrinology (M.B.) from the Institute of Semeiotica Medica; Department of Pediatrics (N.A.G.), University of Padova, Padova, Italy; FIRS Laboratories (B.R.S., J.F., S.C.), RSR Ltd, Parc Ty Glas, Llanishen, Cardiff CF4 5DU and Department of Medicine, University of Wales, College of Medicine, Heath Park, Cardiff CF4 4XN; United Kingdom

Address all correspondence and requests for reprints to: Prof. Corrado Betterle, Istituto di Semeiotica Medica, Cattedra di Immunologia Clinica e Allergologia, Università di Padova, Via Ospedale 105, 35128—Padova, Italy.

Adrenal cortex autoantibodies (ACA) were measured by immunofluorescence in 808 children with organ-specific autoimmune diseases without adrenal insufficiency. ACA were found in 14 children (1.7%), mostly in hypoparathyroidism (48%). Ten ACA-positive and 12 ACA-negative children were followed up for a maximum of 10 yr by evaluation of adrenocortical function (ACTH test) and autoantibody status. In all patients steroid-producing cell autoantibodies were assessed by immunofluorescence and autoantibodies to steroid 21-hydroxylase, 17-hydroxylase, and cytochrome P450 side-chain cleavage enzyme by immunoprecipitation assay. All 10 ACA-positive patients were positive for 21-hydroxylase autoantibodies. Six were positive for steroid-producing cell autoantibodies and 5 also for autoantibodies to 17-hydroxylase and/or P450 side-chain cleavage enzyme. Overt Addison’s disease developed in 9 (90%) ACA/21-OH-antibody-positive children after 3–121 months, and 1 remaining child had subclinical hypoadrenalism. By contrast, all ACA/21-OH antibody-negative children maintained normal adrenal function. Adrenal failure was not related to ACA titres, sex, adrenal function, type of preexisting autoimmune disorder, or human leucocyte antigens D-related status. In conclusion, in children with autoimmune endocrine diseases, ACA/21-hydroxylase autoantibodies are important predictive markers for the development of Addison’s disease.

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