Cardiac tamponade in a patient with autoimmune polyglandular syndrome type 2

Abstract

We describe a case of a 40-year-old woman who was admitted to the intensive care unit with a rapid onset of dyspnea and orthopnea. She presented progressive weakness, weight loss and secondary amenorrhea during last year, while intermittent fever was present for the last two months. Initial biochemical evaluation showed anemia, hyponatremia and increased C-reactive protein levels. Clinical and echocardiographic evaluation revealed cardiac tamponade, which was treated with pericardiocentesis. Pleural fluid samples were negative for malignancy, tuberculosis or bacterial infection. Hormonal and serologic evaluation led to the diagnosis of autoimmune polyglandular syndrome (APS) type 2 (including primary adrenal insufficiency and autoimmune thyroiditis), possibly coexisting with systemic lupus erythematosus. After symptomatic rheumatologic treatment followed by replacement therapy with hydrocortisone and fludrocortisone, the patient fully recovered. In patients with the combination of polyserositis, cardiac tamponade and persistent hyponatremia, possible coexistence of rheumatologic and autoimmune endocrine disease, mainly adrenal insufficiency, should be considered. Early diagnosis and non-invasive treatment can be life-saving.Learning points:In patients with the combination of polyserositis, cardiac tamponade and persistent hyponatremia, possible coexistence of rheumatologic and autoimmune endocrine disease, mainly adrenal insufficiency, should be considered.Early diagnosis and non-invasive treatment can be life-saving for these patients.Primary adrenal insufficiency requires lifelong replacement therapy with oral administration of 15–25 mg hydrocortisone in split doses and 50–200 µg fludrocortisone once daily.

Cardiac Tamponade in Adrenoleukodystrophy with Addison’s Disease

“…Acute pericardial effusion is mainly idiopathic in developed countries, and presumed to be of viral origin although specific causes have not been identified. Although in this case we did not identify a definite aetiology for the cardiac tamponade, an association between cardiac tamponade and concurrent AD has been reported in the literature [2][3][4] . The underlying mechanism for pericardial effusion is possibly autoimmune inflammation of the pericardium, which leads to an acute inflammatory reaction and fluid accumulation [5] .…”

Coexistence of Riehl’s Melanosis, Lupus Erythematosus and Thyroiditis in a Patient

“…Serum adrenal hormones, sex hormones, and blood glucose were all within normal ranges, and the results of adrenal computed tomography scanning were negative, and type II polyglandular autoimmune syndrome was excluded, which could cause hyperpigmentation as skin damage. 9 This suggests that skin hyperpigmentation in this patient was not triggered by thyroid disease.…”