Long-term cardio-metabolic outcomes in patients with classical congenital adrenal hyperplasia: is the risk real?

Abstract

Data on the long-term cardio-metabolic outcomes classical congenital adrenal hyperplasia (CAH) patients have been published with controversial results. Conventional treatment recommends hydrocortisone during childhood; and short and/or long-acting glucocorticoid during adulthood, associated or not with mineralocorticoid, in an attempt to simulate normal cortisol secretion and to normalize androgen excess. However, the balance between glucocorticoid over or undertreatment is very challenging, and patients frequently oscillate between hypercortisolism or hyperandrogenism. Considering these data, we reviewed the frequency of metabolic syndrome components and other cardiovascular risk factors in CAH. Several studies reported increased prevalence obesity, abnormal body composition, increased homeostasis model assessment of insulin resistance and blood pressure levels in CAH patients. However, the evidence quality is still low, because most studies used different glucocorticoid regimes and had heterogeneous goals for hormonal control. Despite the above-mentioned scenario of increased frequency of some cardiovascular surrogate markers in patients, most cohorts comprised young adults, and it is not known if patients will present high frequency of cardiovascular disease in the future. Prospective randomized studies comparing different glucocorticoid regimens should establish the real role of glucocorticoid and androgens on metabolic/cardiovascular profile.