Bilateral Adrenal Infarction, Hypoadrenalism and Splinter Haemorrhages in the ‘Primary’ Antiphospholipid Syndrome

Abstract

We describe a patient with a 3-year history of recurrent deep vein thromboses (DVT) of the lower limbs, who developed adrenal insufficiency following withdrawal of warfarin therapy. Multiple splinter haemorrhages of the nail beds were evident, simultaneous with the development of adrenal infarction in the absence of infective endocarditis. CT scans of the adrenal glands were consistent with bilateral adrenal infarctions. The patient had persistently high titres of IgG anticardiolipin antibodies (aCL) over the previous 4 years in the absence of antinuclear antibodies (ANA), antibodies to double stranded deoxyribonucleic acid (dsDNA) or extractable nuclear antigens (ENA). Thrombocytopenia and an intermittently positive Coombs' test had been noted. Previous episodes of DVT were associated with inadequate warfarin control and a period of warfarin resistance. He conforms to a diagnosis of a 'primary' antiphospholipid syndrome.

Acute Adrenal Failure Associated with Primary Antiphospholipid Syndrome and Homozygosity for MTHFR C677T

“…Ames et al have reported the case of a patient with PAPS who developed AI with adrenal failure following the withdrawal of warfarin therapy (17).…”

Primary Antiphospholipid Syndrome: An Unusual Cause of Adrenal Insufficiency

“…Like lupus anticoagulants, anticardiolipin antibodies were initially described in systemic lupus erythematosus, but now both have also been demonstrated in patients with other autoimmune disorders, in association with drugs and infection [6, 7, 8, 9, 10]. However, many patients with antiphospholipid antibodies have no other underlying disease [1, 2, 4, 11, 12, 13, 14, 15, 16]. Pelkonen et al [3]described deep vein thrombosis associated with lupus anticoagulant and anticardiolipin antibodies in 3 children.…”

“…In 1989, we first noticed an association of primary antiphospholipid syndrome and Addison’s disease in a patient with a history of deep vein thrombosis who developed acute adrenal gland insufficiency [2]. Ames et al [13]reported a patient with a history of deep vein thrombosis who developed acute adrenal insufficiency following withdrawal of warfarin therapy. The CT scans of their patient were thought of show swollen edematous adrenal glands with partial resolution occurring after 4 months, compatible with a previous acute infarction.…”

“…The association of antiphospholipid syndrome with the development of adrenal gland failure has been reported in many patients during the last 10 years, mostly due to bilateral cortical hemorrhage or thrombosis of adrenal vessels [11, 12, 13, 14, 15, 16]. Adrenal failure usually occurs in these patients with the established syndrome and is, therefore, of little diagnostic difficulty.…”

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

“…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.…”