Endocrine Abstracts (2009) 20 P13

Autoimmune adrenal insufficiency: Addison's disease versus idiopathic isolated secondary adrenal insufficiency

Anna Kasperlik-Zaluska1, Barbara Czarnocka2, Wojciech Jeske1, Lucyna Papierska1, Anna-Lena Hulting3, Sophie Bensing3, Patricia Crock4 & Olle Kämpe5


1Department of Endocrinology, Centre for Postgraduate Medical Education, Warsaw, Poland; 2Department of Biochemistry and Molecular Medicine, Centre for Postgraduate Medical Education, Warsaw, Poland; 3Department of Molecular Medicine, Karolinska Institutet, Karolinska Hospital, Stockholm, Sweden; 4John Hunter Children’s Hospital, University of Newcastle, Newcastle, Australia; 5Department of Medical Sciences, University Hospital, Uppsala, Sweden.


Studies on autoimmune Addison’s disease (AD) have a long tradition, while knowledge on idiopathic isolated secondary adrenal insufficiency (SAI), which also seems to be caused by autoimmunity, is not so popular. Our presentation aimed at comparing two groups of patients: 1) 238 patients with AD (183 women, F/M=4.3), aged 8–61 years at time of diagnosis and 2) 301 patients with SAI (269 women, F/M=8.4), aged 17–87 years at time of diagnosis. Maximum time of duration: 44 years in AD, 18 yrs in SAI. Hyperpigmentation in AD was the main clinical sign differentiating the both groups of patients; in AD clinical features were more dramatically manifested. Adrenal crisis was more frequent in AD while in SAI threatening adrenal crisis was not a rare finding. Cortisol levels were low in both groups (lower in AD), however they rose significantly only in SAI after stimulation with synthetic ACTH. High ACTH levels in AD were in contrast with low ACTH values in SAI. Biochemical analyses revealed that hyperkalemia was typical for AD, while hyponatremia was more characteristic for SAI. Additional autoimmune disorders were present in 79% of AD and in 67% in SAI. The main autoimmune diseases in AD: thyroid diseases – 34%, premature ovarian failure (POF) – 14%, IDDM and vitiligo – 13%, while in SAI – 36, 4, 3 and 4% respectively. Thyroid autoantibodies were found in 72% in AD and in 60% in SAI. Adrenal autoantibodies were detected in 55% of 115 AD patients under study, while pituitary autoantibodies in 34% out of 65 patients with SAI. In AD group 33 women became pregnant and delivered healthy children, which contrasted with only one patient with SAI (mainly due to a higher age in SAI). Conclusions: apart of clinical picture and hormonal pattern the main differences between the both groups concerned age at time of diagnosis, F/M ratio, type of electrolyte disturbances, frequency of adrenal crisis, frequency of POF, IDDM and vitiligo.

Supported by a 501-2-2-07-30/02 CMKP Grant.

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