ESPE Abstracts

Background: Primary adrenal insufficiency (PAI) is characterised by the immune destruction of the adrenal cortex and leads to impaired production of the adrenal hormones. It is a potentially life-threatening condition that can be managed if identified early. The onset is insidious and often difficult to diagnose. Screening for adrenal autoantibodies is routinely performed in autoimmune conditions such as type 1 diabetes mellitus (T1DM). The guidance around the management of patients with T1DM following a positive adrenal antibody result is limited.

Objectives: To evaluate our current practice and investigate the outcome of patients with T1DM who have a positive adrenal antibody result.

Design: We identified patients by looking at all adrenal antibody tests performed on paediatric patients at our large tertiary centre between January 2014 and July 2021. We reviewed the electronic record of all patients with a positive result to explore the indication for each test and clinical outcome. We looked retrospectively at all results available for each patient.

Results: There were 2451 adrenal antibody tests performed over this period on 1334 patients. Of these results, 27/2451 (1.1%) were positive, 22/2451 (0.9%) were equivocal, 4/2451 (0.2%) were reported as weak positive and 23/2451 (0.9%) were not processed due to sampling or lab issues. The 27 positive tests identified 22 patients. In 8/22 (36%) the investigations were performed due to clinical suspicion of primary adrenal insufficiency. Conversely, in 14/22 (64%) they were performed as part of a screening process. Thirteen (93%) of these were for routine screening for diabetes and 1/14 (7%) was for monitoring of an autoimmune polyendocrine syndrome. The follow up period from initial positive result for the diabetic patients ranged from 0.69-10.56 years (mean 6.15). Only 1/13 (7.69%) diabetic patient developed primary adrenal insufficiency over this period. The interval between the first positive adrenal antibody result and diagnosis of PAI was 0.22 years. The practice of repeating adrenal autoantibodies and performing short synacthen tests varied between patients.

Conclusion: Our study showed that less than 1% of all adrenal antibody tests reported positive results in paediatric patients undergoing routine screening for diabetes. Of these, only one patient developed PAI and this was after a short interval of less than 3 months. Whilst guidelines for screening for adrenal antibodies exist, there is limited guidance for follow up of those with positive antibodies, especially in T1DM. A standardised approach for these patients would be useful.