Endocrine Abstracts

A 22-year-old lady presented with an acute abdomen and underwent appendicectomy for early appendicitis. Post-operatively she developed headache, dizziness and neck stiffness, and later impaired attention and perseveration. Clinical examination showed papilloedema, a left IVth nerve palsy and left sided cerebellar signs. A CT head scan showed evidence of subarachnoid haemorrhage (SAH) and subsequent MRI confirmed SAH with some meningeal enhancement, as well as intraparenchymal haemorrhage in the cerebellum and postero-superior occipital cortex. However, a four-vessel angiogram showed no aneurysms. CSF analysis failed to show xanthochromia but was consistent with a lymphocytic meningitis. Inflammatory markers were raised (ESR 96 mm/1st hour, CRP 314). She developed hyponatraemia (126 mmol/l) and hyperkalaemia (5.5 mmol/l), and underwent a short synacthen test (250mcg im), which confirmed adrenal insufficiency: baseline cortisol 44 nmol/l, peak response 42 nmol/l. Abdominal CT showed bilaterally swollen adrenal glands and adrenal MRI features consistent with bilateral adrenal haemorrhage. She was commenced on steroid replacement and subsequently made good progress.

Haematological testing revealed deranged clotting times with an APTT of 44 sec and INR of 1.2. Platelets were within the normal range. Russell's viper venom test was consistent with the presence of the lupus anticoagulant. However, anticardiolipin antibodies were negative as were antibodies to beta 2 glycoprotein I. She had a mildly positive ANA titre of 1/320, but negative dsDNA, ENAs and ANCA.

In summary, we describe a case of bilateral adrenal haemorrhage associated with a lupus anticoagulant syndrome (LAS). Although SLE-associated LAS usually presents with prothrombotic disorders, it can be associated with a haemorrhagic illness. However, characterisation of the lupus anticoagulant in this case suggested a secondary aetiology, most likely with infection as the triggering factor. Bilateral adrenal haemorrhage remains an extremely rare cause of acute adrenal insufficiency.