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Polyglandular Autoimmune Syndrome Type-Ii Presented with Acute Renal Injury
( Veysel Ozalper ),( Ibrahim Cetindagli ),( Omer Tekin ),( Yalcin Onem ),( Yusuf Hancerli ),( Eylem Cagiltay ),( Barbaros Basbug ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Introduction: Polyglandular autoimmune syndrome (PAS) is a rare disease, characterized by failure of more than two endocrine glands. Type-II of this syndrome is the most common of the immunoendocrinopathy syndromes and characterized by the obligatory occurrence of autoimmune Addison disease in combination with thyroid autoimmune diseases and/or type-I diabetes mellitus. We report a case of PAS-TypeII presented with prerenal acute renal failure. Case Report: A twenty-two-year-old man had a hyperpigmentation of gingiva within three months, fatigue and amnesia within two months, emesis and vomit within one months, admitted because of persistent vomiting and ten kilogram weight loss. Initial laboratory fi ndings were: urea:87mg/dl, kreatinin:1.31mg/dl, sodium:125mmol/L, potassium: 5.6mmol/L, albumin:4.16g/dl, calcium:123mg/dl. In his arterial blood gas analyse, he had a normal anion gap metabolic asidosis. pH:7.24, HCO3:12.8mEq/L, pCO2:30mm- Hg. He had a prerenal acute renal injury because of persistent vomiting. Other laboratory fi ndings were Hemoglobine:12.9g/dl, TSH:0,004 uIU/ml, FreeT4:2,16ng/dl, Anti-TPO:876 IU/ml, AntiThyroglobulin:9,72IU/ml, Cpeptid:0,27ng/ml, Kortizol:0,1ug/dl, ACTH:22,8pg/ml, HbA1c: %4,7.. A thyroid-scintigraphy showed diffuse homogenous thyroid involvement. His pituitary magnetic-resonance-imaging, thoracoabdominal- tomography and esophagogastroscopy was normal. Based on his fi ndings above and intravenous ACTH stimulation test, adrenal insuffi ency and otoimmune thyroiditis were diagnosed. General condition improved gradually following the administration of hydrocortisone, fl udrocortisone, thyromazol, propranolol. Conclusions: As a conclusion, in persistant vomiting patients, endocrine functions must be performed. A patient who presents with a single autoimmune diseases must be considered at risk of other autoimmune diseases. High index of suspicion, early recognition and treatment can be life-saving, particularly when there is adrenal and thyroid component.
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