Subscribe to RSS
DOI: 10.1055/s-0044-1779745
Schmidt's Syndrome: An Uncommon Cause of Spontaneous Hypoglycemia
Funding None.Abstract
Schmidt's syndrome, or autoimmune polyendocrine syndrome type 2 (APS-2), is an uncommon disorder characterized by the co-occurrence of autoimmune thyroiditis and adrenalitis. APS-2 is defined as a combination of Addison's disease, autoimmune thyroid disease, and/or type 1 diabetes mellitus. It is an autosomal dominantly inherited polygenic disorder with incomplete penetrance; the candidate genes include but are not limited to HLA-DR3, HLA-DR4, CTLA-4, PTPN22, and CD25-IL-2. Autoimmune thyroiditis, often Hashimoto's disease, results in hypothyroidism. Primary adrenal failure results in enhanced secretion of adrenocorticotrophic hormone melanocyte and co-secretion of melanocyte-stimulating hormone, contributing to hyperpigmentation. Mineralocorticoid deficiency results in salt wasting, fatigue and cramps, postural hypotension, and hyperkalemia. Cortisol, an insulin counter-regulatory hormone, plays a pivotal role in maintaining euglycemia; deficiency predisposes to the development of hypoglycemia. We here report a rare presentation of Schmidt's syndrome as hypoinsulinemic hypoglycemia in a middle-aged male patient. Management includes treatment of acute hypoglycemic episodes with glucose or glucagon, long-term glucocorticoids and mineralocorticoids for adrenal insufficiency, and thyroid hormone supplements for hypothyroidism. This case report and brief overview aim to contribute to the scientific understanding of Schmidt's syndrome/APS-2. Additionally, here we briefly outline the diagnostic challenges in hypoglycemia evaluation, including the utilization of Whipple's triad and the gold standard supervised 72-hour fast and evaluation for primary adrenal and thyroid insufficiencies.
Keywords
Schmidt's syndrome - APS-2 - polyendocrine syndrome - Addison's disease - hypoglycemia - hypothyroidismConsent
Consent obtained from the patient for publication without revealing individual identity.
Publication History
Article published online:
27 February 2024
© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
- 1 Carpenter CC, Solomon N, Silverberg SG. et al Schmidt's syndrome (thyroid and adrenal insufficiency). A review of the literature and a report of fifteen new cases including ten instances of coexistent diabetes mellitus. Medicine (Baltimore) 1964; Mar; 43: 153-180
- 2 Kahaly GJ, Frommer L. Polyglandular autoimmune syndromes. J Endocrinol Invest 2018; 41 (01) 91-98
- 3 Singh G, Jialal I. Polyglandular autoimmune syndrome type II. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023. Jan-. Accessed February 2, 2024 at: https://www.ncbi.nlm.nih.gov/books/NBK525992/
- 4 Tincani A, Ceribelli A, Cavazzana I, Franceschini F, Sulli A, Cutolo M. Autoimmune polyendocrine syndromes. In: Shoenfeld Y, Cervera R, Gershwin ME. eds. Diagnostic Criteria in Autoimmune Diseases. Humana Press; 2008.
- 5 Eisenbarth G, Wilson P, Ward F, Lebovitz HE. HLA type and occurrence of disease in familial polyglandular failure. N Engl J Med 1978; 298 (02) 92-94
- 6 Cryer PE. Symptoms of hypoglycemia, thresholds for their occurrence, and hypoglycemia unawareness. Endocrinol Metab Clin North Am 1999; 28 (03) 495-500 , v–vi
- 7 Wester M, Bergmann T, Müller-Schilling M, Maier LS, Sossalla ST. Transient hypoglycemia as a rare cause of recurring transient loss of consciousness: a case report. J Med Case Rep 2021; 15 (01) 261
- 8 Cryer PE, Axelrod L, Grossman AB. et al; Endocrine Society. Evaluation and management of adult hypoglycemic disorders: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2009; 94 (03) 709-728
- 9 Dharmarajan TS, Russell RO, Dabhi K. Recurrent, refractory hypoglycemia presenting as a behavioral disorder in an older woman. J Am Geriatr Soc 1999; 47 (03) 380-381
- 10 Accessed February 2, 2024 at: https://diabetes.org/living-with-diabetes/treatment-care/hypoglycemia
- 11 Michels A, Michels N. Addison disease: early detection and treatment principles. Am Fam Physician 2014; 89 (07) 563-568
- 12 Klubo-Gwiezdzinska J, Wartofsky L. Hashimoto thyroiditis: an evidence-based guide to etiology, diagnosis and treatment. Pol Arch Intern Med 2022; 132 (03) 16222
- 13 Passanisi S, Timpanaro T, Lo Presti D, Caruso-Nicoletti M. Recurrent hypoglycaemia in type-1 diabetes mellitus may unravel the association with Addison's disease: a case report. BMC Res Notes 2014; 7: 634
- 14 Abdullah I, Bdaiwi AS, Wess M, Camferdam RR. Atypical case of Schmidt's syndrome in a young male. Cureus 2022; 14 (06) e26322