Autoimmune Polyglandular Syndrome Type II as a Cause of Recurrent Cardiac Tamponade

ABSTRACT Objective: This study reports an unusual presentation of autoimmune polyglandular syndrome type II (APS-II) with recurrent cardiac tamponade due to pericarditis in a young male patient. Methods: We report clinical presentation, laboratory data, and imaging studies for our patient. Relevant literature is reviewed. Results: Our patient was a 29-year-old Hispanic man with a history of primary hypothyroidism who presented to the emergency department complaining of chest pain and found to have cardiac tamponade due to pericardial effusion. He rapidly became hemodynamically unstable and underwent pericardiocentesis, after which he was admitted to the intensive care unit. The patient gradually recovered and was discharged after 15 days of hospitalization, while the etiology of his illness remained unclear. Four months later, he again presented with similar symptoms and was diagnosed with recurrent cardiac tamponade. He became hemodynamically unstable, and underwent pericardiocentesis. His morning serum cortisol level was found to be undetectable and further workup confirmed autoimmune etiology of primary adrenal insufficiency. The presence of hypothyroidism, vitiligo, and subsequent adrenal insufficiency in the context of positive glutamic acid decarboxylase antibody suggested the diagnosis of APS-II in our patient. Conclusion: APS-II has many associated complications. Cardiac tamponade is a rare presentation of APS-II but it is crucial to recognize it because recurring pericarditis and cardiac tamponade can be fatal in these patients. Accurate diagnosis and timely hormone replacement with monitoring for cardiac complications can prevent fatal outcomes. Abbreviations: APS-II autoimmune polyglandular syndrome type II; ED emergency department; T1DM type 1 diabetes mellitus