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Long-Term Corticosteroid Replacement and Bone Mineral Density in Adult Women with Classical Congenital Adrenal Hyperplasia

Division of Reproductive Endocrinology, Department of Gynecology and Obstetrics (J.A.K., B.J.B., H.A.Z.), and Division of Pediatric Endocrinology, Department of Pediatrics (A.B.W., C.J.M.), Johns Hopkins School of Medicine, Baltimore, Maryland 21287; and Department of Epidemiology (K.A.C.), Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland 21205

Address all correspondence and requests for reprints to: Dr. Jeremy A. King, 2330 West Joppa Road, Suite 301, Lutherville, Maryland 21093. E-mail: jking8{at}mac.com.

Context: Concern has been raised regarding the potential impact of chronic glucocorticoid therapy on the bone mineral density (BMD) of patients with congenital adrenal hyperplasia (CAH).

Objective: The purpose of this investigation was to assess the impact of chronic glucocorticoid replacement in adult women with classical CAH.

Patients and Design: We used dual energy x-ray absorptiometry to evaluate lumbar spine and whole body BMD in 11 women with salt-losing (SL) CAH and 15 with the simple virilizing form. Physical characteristics and serum hormone concentrations were also measured. Results were compared with those of unaffected sisters of CAH patients (n = 9).

Main Outcome Measure: BMD was the main outcome measure.

Results: Osteopenia was noted in 45% of SL CAH patients, 13% of patients with the simple virilizing form, and 11% of controls. Lumbar spine and whole body BMDs of CAH subjects were lower than those of controls (P < 0.05). Compared with CAH subjects with normal BMD, those with osteopenia had reduced serum levels of dehydroepiandrosterone sulfate and dehydroepiandrosterone. Adrenal androgen levels were particularly suppressed among postmenopausal women receiving glucocorticoid replacement.

Conclusions: Adult women with classical CAH treated with long-term glucocorticoids are at risk for decreased BMD, especially those with the SL form. Oversuppression of adrenal androgens is associated with increased risk for bone loss in this population.

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