Endocrine Abstracts

Background: Primary adrenal insufficiency due to adrenal hemorrhage from anti-phospholipid syndrome (APS) is an uncommon and life threatening disorder. We present a case, highlighting the challenges in the diagnosis and management of this disorder.

Case presentation: A 59-year-old Chinese lady presented with a 2-year history of bilateral flank pain, lethargy, poor appetite and weight loss. Initial blood investigations were unremarkable except for a cholestatic liver function test. A computed tomography of the abdomen incidentally revealed bilateral adrenal hemorrhages. She had not sustained any trauma, did not have a bleeding disorder and was not taking any anticoagulants. Subsequent workup confirmed the diagnosis of primary APS with elevated levels of lupus anticoagulant, anticardiolipin and anti-B2 glycoprotein. She did not have any other associated connective tissue disorders or malignancies. Thrombophilia screen was normal. There was concomitant primary adrenal insufficiency with a blunted synacthen test response. Cortisol was 35 nmol/l at 0 min and 34 nmol/l 30 min after synacthen was given; ACTH was markedly elevated at 194 pmol/l. Aldosterone was undetectable while renin was elevated at 4.96 ng/ml per hour. She was started on hydrocortisone and fludrocortisone in addition to anticoagulation. Interval adrenal imaging showed near complete resolution of adrenal hemorrhage but the adrenal insufficiency persists with blunted synacthen test and undetectable aldosterone despite 1 year of follow up.

Conclusion: Clinicians need to have a heightened awareness of the association between adrenal hemorrhage and APS and institute treatment early. Despite radiological resolution of the adrenal hemorrhage, adrenal insufficiency persists, likely secondary to irreversible hemorrhagic infarction of adrenal tissue.