Polyglandular autoimmune syndrome Type II in patients with idiopathic Addison's disease

• Published in: Acta endocrinologica (Copenhagen) Vol. 122; no. 4; pp. 472 - 478
• Main Authors: Papadopoulos, K I, Hallengren, B
• Format: Journal Article
• Language: English
• Published: Denmark 01.04.1990
• Subjects: Addison Disease - complications; Adolescent; Adult; Autoimmune Diseases - complications; Autoimmune Diseases - epidemiology; Child; Diabetes Mellitus, Type 1 - complications; Diabetes Mellitus, Type 1 - epidemiology; Female; Humans; Male; Middle Aged; Prevalence; Retrospective Studies; Thyroid Diseases - complications; Thyroid Diseases - epidemiology; Thyroid Diseases - immunology; Time Factors
• ISSN: 0804-4643; 0001-5598; 1479-683X
• DOI: 10.1530/acta.0.1220472

The frequency of polyglandular autoimmune syndrome Type II, (idiopathic or autoimmune Addison's disease associated with autoimmune thyroid disease, and/or insulin-dependent diabetes mellitus), was retrospectively investigated in 44 patients (26 females and 18 males) with idiopathic Addison's disease (median age at onset 32.5 years, range 8–62; median observation time 17 years, range 0.5–41) evaluated between 1966 and 1988 in the Department of Endocrinology, General Hospital, Malmö. Twenty-two patients (16 females and 6 males) fulfilled the criteria for polyglandular autoimmune syndrome Type II and of these 16 had autoimmune thyroid disease and 9 insulin-dependent diabetes mellitus. In 7 of 9 patients insulin-dependent diabetes mellitus developed prior to Addison's disease, whereas autoimmune thyroid disease was diagnosed before as well as after Addison's disease and with a similar age distribution as Addison's disease. In conclusion, the present study indicates that polyglandular autoimmune syndrome Type II occurs in about 50% of patients with idiopathic Addison's disease and that autoimmune thyroid disease is more common than insulin-dependent diabetes mellitus in these patients. The present study also indicates that insulin-dependent diabetes mellitus precedes Addison's disease in a majority of these patients and usually develops at a young age, whereas autoimmune thyroid disease precedes as well as succeeds Addison's disease and occurs at all ages in these patients.