Secondary Adrenal Insufficiency and Primary Antiphospholipid Syndrome

Abstract

A woman with isolated ACTH deficiency and empty sella in a background of primary antiphospholipid syndrome (APS) is described. A 24-year-old woman was admitted for premature delivery at 30 weeks of gestation and was found to have severe pulmonary hypertension and right sided heart failure. A few hours after delivery, without excessive blood loss, she suddenly lost consciousness, and was found to be hypotensive and hypoglycemic. The findings on hormonal evaluation were consistent with isolated secondary adrenal insufficiency as both ACTH and cortisol levels were very low. Magnetic resonance imaging of the pituitary fossa showed an empty sella. Her severe cor pulmonale was found to be due to primary APS. The patient was given replacement of glucocorticoid and treated with frusemide, spironolactone, warfarin, sildenafil and inhaled iloprost for her pulmonary hypertension, after which her clinical status improved substantially. Follow-up tests of the hypothalamic-pituitary axis found normal serum LH, FSH, TSH, FT4 and prolactin secretion; serum ACTH and plasma cortisol levels remained low.