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Endocrine Abstracts (2024) 99 EP1301 | DOI: 10.1530/endoabs.99.EP1301

ECE2024 Eposter Presentations Late Breaking (127 abstracts)

Autoimmune polyglandular syndrome type 2: a clinical case of a rare combination of primary adrenal insufficiency and latent autoimmune diabetes in adults

Ilana Katsobashvili , Laura Ebanoidze , Ivan Golodnikov , Larisa Dzeranova , Ekaterina Pigarova & Tatyana Nikonova


Endocrinology Research Centre, Moscow, Russian Federation


Introduction: According to statistical data, the combination of chronic adrenal insufficiency and diabetes mellitus is the rarest among patients with autoimmune polyglandular syndrome type 2 (APS-2).

Clinical Case: In 2017, a 32-year-old patient B. with excess body weight manifested type 2 diabetes mellitus with typical clinical symptoms of carbohydrate metabolism disorder. Metformin and gliclazide were recommended. After a viral pneumonia episode with hospitalization in the intensive care unit in December 2022, the patient experienced pronounced weakness, nausea, hypotension, and weight loss. During outpatient examination for worsening general well-being, a sharp increase in ACTH and a decrease in blood cortisol were detected for the first time. In May 2023, the patient was urgently hospitalized in the endocrinology department, where primary adrenal insufficiency was diagnosed based on hormonal studies (morning blood cortisol – 94 nmol/l, ACTH – 1250 pg/ml). Hydrocortisone 10 mg in the morning/evening and fludrocortisone 0.1 mg/day were prescribed. Due to organizational difficulties, mineralocorticoid therapy was not administered. Due to decompensated carbohydrate metabolism (HbA1c - 19%), the patient was switched to basal-bolus insulin therapy (Protaphane 8 IU in the morning, 6 IU in the evening, Biosulin R 4 IU before main meals). In November 2023, during hospitalization in an endocrinological ward, considering a low C-peptide level of 0.501 ng/ml (1.1-4.4), antibodies to GAD >2000 U/ml (0-10), insulin – 6.38 U/ml (0-10), and tyrosine phosphatase <1 U/ml (0-10), latent autoimmune diabetes in adults (LADA) was confirmed in combination with other criteria (age >35 years, being on oral hypoglycemic therapy for >6 months). Hormonal studies revealed morning ACTH - 1044 pg/ml (7.2-63.3) and morning cortisol - 45.5 nmol/l (171-536). The hydrocortisone regimen was adjusted to 10 mg in the morning, 5 mg at 13:00, and 5 mg at 18:00. Data indicating mineralocorticoid deficiency were obtained: sodium - 133.7 mmol/l (136-145), potassium - 4.09 mmol/l (3.5-5.1), renin - 75.95 mU/l (2.8-39.9), and fludrocortisone 0.1 mg/day was initiated. To exclude celiac disease, antibodies to gliadin IgA were taken, yielding 2.79 U/ml (0-12). Upon admission, HbA1c was 14%, and insulin therapy was optimized: insulin degludec 100 U/ml 12 IU at 22:00, insulin aspart 6 IU before main meals, stabilizing glycemic indicators within individual target ranges.

Conclusion: The clinical manifestations of APS-2 underscore the complex nature of the interplay between autoimmune and metabolic disorders necessitating an interdisciplinary approach in the timely diagnosis and treatment of this syndrome.

Volume 99

26th European Congress of Endocrinology

Stockholm, Sweden
11 May 2024 - 14 May 2024

European Society of Endocrinology 

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