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 insuf-ficiency 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. (AACE Clinical Case Rep. 2017;3:e353-e356) Abbreviations: APS-II = autoimmune polyglandular syndrome type II; ED = emergency department; T1DM = type 1 diabetes mellitus e354 APS-II and Cardiac Tamponade, AACE Clinical Case Rep. 2017;3(No. 4)

A Rare Case of Addison’s Disease Presenting With Intermittent Pancytopenia and Cardiac Tamponade

“…13 Pericarditis with cardiac tamponade has been described in Addison's disease occurring in the setting of autoimmune polyglandular syndrome type II(PGA-II) in approximately 15 cases. [14][15][16][17][18][19] Similar to viral pericarditis, the proposed mechanism in J o u r n a l P r e -p r o o f Addison's is that autoimmune inflammation of the pericardium leads to an acute inflammatory reaction and rapid fluid accumulation. Immune complexes and antibodies have also been found in fluid samples, supporting autoimmune inflammation in the pathogenesis of pericarditis.…”