Introduction: Patients with primary adrenal insufficiency (Addisons disease) and patients with congenital adrenal hyperplasia (CAH) still tend to receive more glucococorticoids than the normal endogenous production in healthy subjects. CAH patients start glucocorticoid treatment usually with diagnosis in their early childhood, whereas Addisons patients have a later onset of their disease and start of their treatment.
Objective: To compare patients with Addisons disease and CAH in regard to their bone mineral density (BMD), the duration of glucocorticoid therapy and the impact of glucocorticoid pharmacogenetics.
Design, setting and participants: In a cross-sectional study patients from one university endocrine outpatient clinic were included (84 patients with Addisons disease, 42 patients with CAH). Bone mineral density (BMD) was measured using DXA scan. Blood samples were analysed for bone markers and 24 h urinary samples were analyzed for bone resorption markers.
Results: Patients with Addisons disease were significantly older (55.1±15.2 vs 39.9±13.8y, P<0.001) and taller (168±10 vs 160±11 cm, P<0.001) than CAH patients, but showed no difference in BMI. Time since diagnosis was shorter in Addisons patients (14.9±10.4 vs 30.5±13.5y, P<0.001). The calculated hydrocortisone equivalent glucocorticoid dose per body surface was higher in CAH than Addisons patients (15.6±7.2 vs 11.9±2.4 mg/m2, P<0.001). No differences were seen in serum Ca, aP, ostase, PTH, 25-vitamin D3, 1,25-vitamin D3, osteocalcin or urinary cross-links. Femoral neck BMD Z-scores were significantly lower in patients with CAH compared to patients with Addisons disease (−0.59±1.17 vs 0.0±0.99, P<0.01); also Z-scores for femoral Wards region were lower in CAH patients (−0.96±1.04 vs −0.28±1.03, P<0.005). No difference was found in lumbar spine Z-scores.
Conclusions: BMD at femoral neck and femoral Wards region were lower in CAH than Addisons disease patients, indicating undesirable effects of higher glucocorticoid dose, usage of longer acting glucocorticoids, and longer duration of replacement therapy.