Primary antiphospholipid syndrome presenting as hyponatremic hypertensive syndrome and acute adrenal insufficiency following it: a rare case

Abstract

Hyponatremic hypertensive syndrome (HHS) is rare and is characterized with hypertension together with hyponatremia, hypokalemia and low plasma osmolarity and considered to be based on renal ischemia. It can rarely develop due to antiphospholipid syndrome (APS). We reported a case of 43-year-old man with unilateral renal artery stenosis due to primary APS. He presented with a hypertensive hyponatremic episode. First, he was treated with antihypertensive drugs. Further, radiological imaging showed enlargement of bilaterally adrenal glands that mimic adrenal masses. Unilateral nephrectomy and adrenalectomy were performed. Pathological examination revealed necrosis in the adrenal tissue and thrombosis in its artery. After operation, acute adrenal insufficiency developed in the patient because of hemorrhagic infarction of bilaterally adrenal glands. He improved after intravenous hydrocortisone treatment. His therapy was maintained with oral prednisolone and fludrocortisone. This patient with HHS, due to primary APS, is the first case in literature and acute adrenal insufficiency is a rare complication of APS.