Development of Graves' hyperthyroidism caused an adrenal crisis in a patient with previously unrecognized non-classical 21-hydroxylase deficiency

Abstract

A 75-year-old woman was found to be unconscious in hospital. She was febrile with a temperature of 38.4 °C. She had hypotension (blood pressure 80/40 mmHg) with serum Na 132 mEq/L and K 5.7 mEq/L (serum Na/K = 23.2), and serum cortisol 0.91 μg/dL, indicative of adrenal failure. She was admitted for the treatment of Graves' hyperthyroidism, and was found to be unconscious in hospital. We encountered a patient with unrecognized adrenocortical disease, in whom development of Graves' hyperthyroidism caused an adrenal crisis. The ACTH stimulation test indicated that she had 21-hydroxylase deficiency (21OHD); after ACTH stimulation, 17-OH-progesterone increased from 0.6 to 10.4 ng/mL (17.3 times), and 17-OH-progesterone/cortisol from 0.0049 to 0.045 (9.2 times). She did not have clinical signs of classical 21OHD. She had non-classical 21OHD (NC21OHD). Development of Graves' hyperthyroidism caused an adrenal crisis in a patient with previously unrecognized NC21OHD. A patient with unrecognized adrenocortical disease developed Graves' hyperthyroidism, which induced an adrenal crisis. She had NC21OHD. © 2010 The Japanese Society of Internal Medicine.