Polyglandular Autoimmune Syndrome, Type 2

JONATHAN LEOR, DAVID LEVARTOWSKY, CHARLOTTE SHARON
1989 Southern medical journal (Birmingham, Ala. Print)  
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
more » ... d 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.
doi:10.1097/00007611-198903000-00024 fatcat:xwe2tddhkvbkjpjx3iwpz352ly