Pericarditis with Cardiac Tamponade and Addisonian Crisis as the Presenting Features of Autoimmune Polyglandular Syndrome type II: a Case Series

Abstract

To present 3 cases of cardiac tamponade and addisonian crisis as the presenting features of autoimmune polyglandular syndrome (APS) type II. We present the clinical features, physical examination findings, radiographic findings, and laboratory investigations in 3 patients and provide an overview of the published cases of pericarditis and APS type II found in a MEDLINE search. Patient 1, a 34-year-old woman, and Patient 3, a 35-year-old woman, had known autoimmune thyroid disease. Patient 2 was a 58-year-old man with no known history of autoimmune disease. All presented with a history of long-standing lethargy, nausea, weight loss, dyspnea, chest pain, and striking hyperpigmentation. Physical examination, laboratory, and radiographic findings were consistent with cardiac tamponade and addisonian crisis. In all 3 patients, serum cortisol was either very low or undetectable with a subnormal response to cosyntropin (250 mcg) stimulation. Cells in the pericardial fluid were predominantly neutrophils, and findings from cytology, microbiology, and serology investigations were normal. Symptoms and pericardial fluid resolved during treatment with high-dose glucocorticoids and various anti-inflammatory agents. Over the follow-up period of at least 2 years, all 3 patients experienced recurrent pericarditis and/or pleuritis despite replacement doses of glucocorticoids, which resolved with high-dose anti-inflammatory therapy. Pericarditis is a rare, but life-threatening manifestation of APS type II that is currently not considered to be a key manifestation of the syndrome. Our experience suggests that APS should be considered in the differential diagnosis for patients presenting with unexplained pericarditis. In patients with established APS, one should be vigilant for the development of recurrent pericarditis.