Endocrine Abstracts

Primary adrenal failure is a rare disorder with a prevalence in developed countries of 93–140 per million and an incidence of 4.7–6.2 per million. Autoimmune adrenalitis is the main cause (80%) followed by tuberculosis (15%). The remaining 5% of cases represent rare disorders with bilateral adrenal haemorrhagic infarction secondary to primary antiphopholipid syndrome being very rare (0.5%).

A 57-year-old female presented with a 3-week history of abdominal pain, nausea, vomiting and a fever. She had previously been diagnosed with primary antiphopholipid syndrome after presenting with transient ischaemic attacks and a cerebrovascular accident. Lupus anticoagulant and anticardiolipin antibodies of both IgG and IgM isotypes were positive. She was anticoagulated with warfarin. Examination found her to be unwell with pyrexia up to 38.9 °C, hypotension and tachycardia. A urinary tract infection was suspected and treated with antibiotics. Lack of clinical response lead to extensive differential diagnoses with none confirmed. Abdominal CT scan coincidentally revealed enlarged adrenal glands with evidence of haemorrhagic infarction. Investigations confirmed hypoadrenalism with 0900 h cortisol 80 nmol/l and ACTH 229.2 ng/l. Short Synacthen test: cortisol levels 0 min 85, 30 min 80 and 60 min 76 nmol/l. Hydrocortisone replacement resulted in rapid clinical improvement.

In most reported cases the diagnosis of haemorrhagic infarction of the adrenal glands in antiphopholipid syndrome was serendipitous as it was in this patient. The presentation of the case was typical of reported cases with fever (65%), abdominal pain (70%), hypotension (60%) and infection as the precipitating factor (40%). Whilst adrenal insufficiency is reported in only 0.4% of patients with antiphopholipid syndrome the typical symptoms and signs offer the potential for educated rather than chance diagnosis.