Endocrine Abstracts (2017) 50 P012 | DOI: 10.1530/endoabs.50.P012

It's a matter of time: the emergence of autoimmune polyendocrine syndrome in autoimmune Addison's disease reaches 100% among those diagnosed more than 50 years ago

Katherine White & Alick Mackay


Addison’s Disease Self-Help Group, Hertford, UK.


Addison’s disease (AD) has an estimated prevalence of 126–144 per million among Northern European populations. Average age at diagnosis is 39, but AD may be diagnosed from pre-teens to 80 year olds.

AD may occur in isolation or as part of autoimmune polyendocrine syndromes. Previous studies identified significant associations with autoimmune thyroid disease (predominantly hypothyroidism), type 1 diabetes mellitis, vitiligo, vitamin B12 deficiency and coeliac disease.

We examined self-reported rates of associated endocrine and autoimmune conditions among British Autoimmune Addison’s Disease (AAD) patients, all members of the Addison’s Disease Self-Help Group (n=444). Co-conditions were analysed by years since diagnosis, with a range from 50 years post-diagnosis. The average years since diagnosis was 12.2. Cohort sizes for advancing decades post-diagnosis were small, making significance tests problematic. Rates of associated conditions generally increased over time, although smaller cohorts were not consistently progressive.

Thyroid conditions increased steadily among each post-diagnosis cohort, to reach 100% of those diagnosed >50 years ago. Type 1 diabetes peaked at 26% among the 31–40 year cohort. Coeliac disease, vitamin B12 deficiency and vitiligo peaked at 10%, 20% and 30% respectively among those diagnosed 41–49 years ago. Premature ovarian/testicular failure peaked at 20% of those diagnosed >50 years ago. (See chart).

Larger Swedish and German studies found broadly similar proportions of AAD patients with thyroid disease. Both had higher rates of insulin-dependent diabetes; rates of B12 deficiency in Sweden were also significantly higher.

Table 1
ConditionBritain (n=444)Sweden (n=1305)Germany (n=2715)P value UK-Sweden
Thyroid48%47%55%0.666
T1Diabetes6%14%8%1
B12 deficiency9%18%1

These findings are a reminder that AAD patients require lifelong monitoring for the development of co-conditions. Endocrinologists should assume that AAD patients are likely to develop a thyroid condition at some stage, and that AAD should be managed as potentially an autoimmune polyendocrine syndrome, even when it is diagnosed in isolation.

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