Endocrine Abstracts

Introduction: On average, 40 to 50% of the patients with autoimmune adrenocortical insufficiency will eventually develop an autoimmune polyglandular syndrome (APS). Our aim was to characterize a population with primary adrenocortical insufficiency (AI) and determine the prevalence of other autoimmune disorders that might establish the diagnosis of APS.

Materials and methods: We included patients with primary AI under surveillance at our Department. Those with iatrogenic causes for AI were excluded. The referred population was characterised on what concerns to gender, age, disease’s duration, familial history of AI and aetiology. We assessed autoimmune markers for Addison disease, type 1 diabetes, gastritis, thyroiditis and celiac disease in those without an obvious cause for AI. Positive markers triggered evaluation of associated dysfunction.

Results: Thirty patients (63.3% females) were included. Mean age- 46.9±15.9; disease’s duration- 19.9±12.1 years. Familial history of AI was present in 23.3% of the cohort. On what concerns to AI aetiology, 63.3% were autoimmune, 20% congenital adrenal hyperplasia, 6.7% X-linked adrenoleukodystrophy, 3.3% tuberculosis, 3.3% antiphospholipid antibody syndrome and 3.3% undetermined. Considering the subgroup of autoimmune AI, 31.5% had at least one positive diabetes autoantibody (40% already diagnosed as having diabetes), 42.1% displayed autoimmune gastritis markers (62.5% with hypergastrinemia, 25% iron deficiency and 12.5% anaemia). Positive thyroid autoantibodies were present in 63.2% of the patients; 75% of them had thyroid dysfunction (hypothyroidism 88.9%). Celiac disease markers were not detected in this population.

Enough criteria for an APS diagnosis were fulfilled in 73.7% patients (85.7% APS type 2; 14.3% APS type 4).

Conclusion: The main aetiology for AI was autoimmune. The commonest associated autoimmune disorders were thyroiditis (63.2%) and gastritis (42.1%).

An early identification of other autoimmune disorders in patients with autoimmune AI will ensure an adequate treatment and follow-up, improving their quality of life. Therefore, a regular screening for autoimmunity is advisable.