Polyglandular Autoimmune Syndrome Type 2 Presenting With Ventricular Tachycardia

Olusegun Sheyin^1, , Taiwo Falade¹ and Olufemi Fasanmade¹

¹Department of Medicine, Lagos University Teaching Hospital, Lagos Nigeria

Cite this paper

Olusegun Sheyin, Taiwo Falade and Olufemi Fasanmade. Polyglandular Autoimmune Syndrome Type 2 Presenting With Ventricular Tachycardia. American Journal of Medical Case Reports. 2014; 2(10):204-205. doi: 10.12691/AJMCR-2-10-2

Abstract

Introduction: Polyglandular autoimmune syndrome (PGA) describes a condition where dysfunction of two or more endocrine glands occurs in association with circulating organ-specific antibodies directed against the involved glands. Case Presentation: A 36 year old female, known type 1 diabetic presented with a 3 week-history of heat intolerance and intermittent palpitations, associated with dyspnea and light-headedness. On examination, a cachexic, lethargic woman with an unrecordable pulse and blood pressure was found. She was found to have sustained monomorphic ventricular tachycardia on cardiac monitor at a rate of 186 beats per minute, confirmed on 12-lead electrocardiogram. The patient was immediately cardioverted, with restoration of sinus rhythm and pulse. Further examination revealed generalized hyperpigmentation with patches of depigmentation, alopecia, a goiter, proptosis and lid lag. Thyroid function test was consistent with thyrotoxicosis and co-syntropin test confirmed adrenal insufficiency. Complete blood count was significant for a macrocytic anemia; with low level of vitamin B12 on testing. A diagnosis of sustained ventricular tachycardia in a patient with PGA type 2 was made and the patient was admitted to the intensive care unit. She was treated with hydrocortisone, fludrocortisone, carbimazole, propanolol and cyanocobalamin. The patient was maintained on amiodarone for one week and she remained in sinus rhythm for the remainder of her hospitalization. Discussion: PGA type 2 is characterized by the occurrence of adrenal insufficiency with thyroid autoimmune disease (hypo or hyperthyroidism) and insulin-dependent diabetes mellitus. Electrolyte abnormalities are frequently found in adrenal insufficiency and may predispose to arrhythmias on a background of increased adrenergic effect of thyrotoxicosis. Conclusion: The presence of an immunoendocrinopathy warrants the search for other endocrine hypofunction. In situations where a life-threatening arrhythmia is present, urgent identification and treatment of the arrhythmia is top priority.

Keywords

adrenal insufficiency, autoimmune, polyglandular, thyrotoxicosis, ventricular tachycardia

Copyright

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References

[1]	Leshin M. Southwestern internal medicine conference: Polyglandular autoimmune syndromes. Am J Med Sci 1985; 290: 77-78.
[2]	Neufeld M, Maclaren NK, Blizzard RM. Two types of autoimmune Addison's disease associated with different polyglandular autoimmune (PGA) syndromes. Medicine (Baltimore) 1981; 60: 355.
[3]	Dickstein G, Shechner C, Nicholson WE, et al. Adrenocorticotropin stimulation test: effects of basal cortisol level, time of day, and suggested new sensitive low dose test. J Clin Endocrinol Metab 1991; 72: 773.
[4]	Crowley S, Hindmarsh PC, Honour JW, Brook CG. Reproducibility of the cortisol response to stimulation with a low dose of ACTH(1-24): the effect of basal cortisol levels and comparison of low-dose with high-dose secretory dynamics. J Endocrinol 1993; 136: 167.
[5]	Oelkers W, Diederich S, Bähr V. Diagnosis and therapy surveillance in Addison's disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone. J Clin Endocrinol Metab 1992; 75: 259.
[6]	Moss AJ. Long QT Syndrome. JAMA 2003; 289: 2041.
[7]	El-Sherif N, Turitto G. Torsade de pointes. Curr Opin Cardiol 2003; 18: 6.
[8]	Passman R, Kadish A. Polymorphic ventricular tachycardia, long Q-T syndrome, and torsades de pointes. Med Clin North Am 2001; 85: 321.