AUTOIMMUNE POLYGLANDULAR SYNDROME ON A COHORT OF PATIENTS WITH PRIMARY ADRENOCORTICAL INSUFFICIENCY

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 50-71-HPA Axis
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-62
Sofia Gouveia1, Cristina Ribeiro2, Márcia Alves2, Joana Saraiva2, Carolina Moreno2, Daniela Guelho2 and Francisco Carrilho*2
1Coimbra's University Hospital, Coimbra, Portugal, 2Coimbra’s University Hospital, Coimbra, Portugal
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 characterized on what concerns to gender, age, disease’s duration, familial history of AI and etiology. 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 etiology, 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% anemia). 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 etiology 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.

Nothing to Disclose: SG, CR, MA, JS, CM, DG, FC

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm