Cardiac Tamponade as Initial Presentation of Autoimmune Polyglandular Syndrome Type 2

Abstract

ABSTRACT: Objective: Autoimmune polyglandular syndrome (APS) involves autoantibodies against multiple endocrine glands causing either hormonal excess or insufficiency. APS type 2, or Schmidt syndrome, is rare and usually seen in middle-aged females and is characterized by the presence of at least 2 of the following: Addison disease, autoimmune thyroid disease, and type 1 diabetes mellitus. In 2008, there were 3 case reports of APS type 2 presenting as pericarditis with cardiac tamponade, 2 of which had recurrent pericarditis.Methods: We present a rare case of APS type 2 presenting as acute pericarditis leading to cardiac tamponade in a young male.Results: A 21-year-old male who presented with chest pain was found to have acute pericarditis. He developed cardiac tamponade with an ejection fraction of 20% and he underwent emergent pericardiocentesis. Clinical tests revealed low sodium (131 mmol/L), normal potassium (4.5 mmol/L), high thyroid-stimulating hormone (17.9 μU/mL), and normal free thyroid hormones. His cortisol was low (0.5 μg/dL). Thyroid peroxidase antibodies were positive (985 U/mL), as was his anti-thyroglobulin antibody (27.2 U/mL). He also had positive 21-hydroxylase antibodies (11.8). His cardiac status responded well to high-dose intravenous steroids and intravenous immunoglobulin. He was discharged stable, however 5 weeks after discharge the patient experienced recurrent pericarditis with pericardial effusion that developed into cardiac tamponade. This time the patient underwent placement of a pericardial window.Conclusion: The patient has APS type 2 as he has Addison disease and Hashimoto thyroiditis. Although rare, it has been reported that acute pericarditis leading to cardiac tamponade can be the initial presentation of APS type 2.Abbreviation: APS autoimmune polyglandular syndrome