Definitive Adrenal Insufficiency Due to Bilateral Adrenal Hemorrhage and Primary Antiphospholipid Syndrome

Abstract

ANTIPHOSPHOLIPID syndrome is characterized by multiple and recurrent venous and arterial thromboses accompanied by and probably caused by circulating antibodies directed against a heterogenous group of phospholipids (1–3). Antiphospholipid syndrome has been described in a large variety of conditions such as connective tissue disorders, malignancies, or some prolonged treatments, but has also been recognized in the absence of all those conditions, leading to the concept of primary antiphospholipid syndrome (4). Contrary to neurological, cardiac, hepatic, skin, or renal manifestations associated with antiphospholipid syndrome, which have been extensively described and commented, the adrenal manifestations of the antiphospholipid syndrome have received scare attention or are completely omitted in large series (1, 5, 6). In patients with primary antiphospholipid syndrome, spontaneous bilateral adrenal hemorrhage is an uncommon condition that may lead to acute adrenal insufficiency and death. It was almost exclusively a postmortem diagnosis before computed tomography (CT) scan was developed. Anecdotal cases of survival have recently been reported in patients with bilateral adrenal hemorrhage associated with the antiphospholipid syndrome (7, 8). We describe a new observation of definitive adrenal insufficiency caused by bilateral adrenal hemorrhage in a woman with a primary antiphospholipid syndrome.