Abstract
Introduction: Antiphospholipid syndrome (APS) is the association of anti-cardiolipin antibodies and venous & arterial thrombosis. APS accounts for <0.5% of cases of hypoadrenalism. We present a series of 5 such cases occurring at our institution plus a further patient identified with unilateral adrenal haemorrhage. Case 1 presented with nausea, vomiting and weight loss. Lupus anticoagulant positive. Commenced aspirin 300 mg. 6-weeks later represented with dehydration, confusion and hyperpigmented skin. Abdominal CT showed bilateral adrenal infarction. Adrenal antibody negative. Peak cortisol 213nmol/L on short synacthen test (SST). Cases 2&3 were known APS but later developed adrenal insufficiency peri-partum. In Case 2, this occurred 3-years following unilateral adrenal haemorrhage. At 6 weeks gestation, warfarin was switched to clexane. 1-week later she developed hydronephrosis and sepsis. CT showed enlarged contralateral adrenal. Over subsequent days ACTH rose to 147 ng/L. Case 3 received clexane during pregnancy. At 30 weeks she underwent urgent caesarean section which was complicated by postoperative wound infection and sepsis. ACTH 321 ng/L. Adrenal infarction on CT abdomen. Cases 4&5 also had pre-existing APS. Case 4 developed GI bleeding on warfarin - corrected with vitamin K and fresh frozen plasma. Subsequently developed femoral DVT, splinter haemorrhages, fever and raised ESR. CT showed bilateral adrenal enlargement. ACTH 321 ng/L, peak cortisol 95 nmol/L on SST. Case 5 acutely developed diarrhoea & vomiting with INR >10 and Hb 4.9 g/dL. Pelvic haematoma on admission CT. Subsequently became septic & hypotensive. Repeat CT showed adrenal haemorrhage. ACTH 290 ng/L, cortisol <30 nmol/L. Case 6 has APS and an adrenal incidentaloma that developed haemorrhagic features 1-year after warfarin therapy. Glucocorticoid production is currently preserved. Conclusion: Acute events such as sepsis or pregnancy can precipitate adrenal failure in patients with APS by venous infarction with or without intraglandular haemorrhage. The presence of unilateral adrenal infarction/haemorrhage may leave a patient vulnerable to later hypoadrenalism.