Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome
A 44-year-old female patient presented to us with acute abdominal pain associated with giddiness and vomiting. She had a very low blood pressure (80/50 mm of Hg). Systemic examination was normal. Her initial serum cortisol was 20 μg/dl (normal 5-25 μg/dl) [].\nRepeat serum cortisol done 24 h later was 1.3 μg/dl and concurrent plasma adrenocorticotropin hormone was 698 pg/ml (normal 45 pg/ml). These feature confirmed the diagnosis of evolving acute adrenal insufficiency (AAI).\nComputed tomography (CT) scan of the abdomen revealed, bilateral enlarged adrenal glands, the right adrenal showed good enhancement with contrast, whereas the left adrenal showed no contrast enhancement suggesting acute ischemia []. CT Angiography repeated at 48 h showed that the previously enhancing right adrenal also failed to enhance with intravenous (IV) contrast. These findings indicated venous infarction of both adrenal glands [].\nIn view of the acute onset of adrenal insufficiency in an otherwise healthy female we initially considered the possibility of underlying vasculitis. This was however ruled out on subsequent evaluation. Thrombotic work-up revealed prolonged activated thromboplastin time, which didn’t correct with the addition of normal plasma, indicating the presence of a circulating anticoagulant. Her prothrombin time was normal, lupus anticoagulant was positive and anti-cardiolipin antibody level was mildly elevated, 30 GPL units (normal <25 GPL units). Protein C and protein S levels were normal. Based on the above clinical and laboratory findings, this patient was diagnosed to have APS with bilateral infarction of the adrenal glands leading to AAI.\nShe was initially treated with IV steroids and heparin infusion. She was subsequently started on the oral warfarin and the dose was titrated to maintain the international normalized ratio between 2.5 and 3.0. At discharge, her abdominal pain had subsided and her blood pressure had stabilized. She was advised to continue tab. hydrocortisone, tab. fludrocortisone and tab. warfarin. At 3 month follow-up, she remained well.