Glucocorticoid remediable aldosteronism (GRA), also describable as aldosterone synthase hyperactivity, is an autosomal dominant disorder in which the increase in aldosterone secretion produced by ACTH is no longer transient. Patients with GRA may be asymptomatic, but the following symptoms can be present:
• High blood pressure
• Intermittent or temporary paralysis
• Muscle spasms
• Muscle weakness
• Metabolic alkalosis
HOW IS GLUCOCORTICOID-REMEDIABLE ALDOSTERONISM DIAGNOSED?
The diagnosis of GRA is supported by dexamethasone suppression testing (DST). Although most patients show a significant improvement in blood pressure levels following DST, PA levels or aldosterone excretion rates have been the primary end points of this test. A fall in aldosterone to nearly undetectable levels after low-dose DST (0.5 mg dexamethasone orally every 6 h over 2–4 days) in GRA is expected and reflects the sole control of aldosterone by ACTH in this disorder. A recent study concluded that a post-DST PA below 4 ng/dL will correctly diagnose GRA patients with high sensitivity and specificity. Gene testing is also done to diagnose GRA.
HOW IS GLUCOCORTICOID-REMEDIABLE ALDOSTERONISM TREATED?
In GRA, the hypersecretion of aldosterone and the accompanying hypertension are remedied when ACTH secretion is suppressed by administering glucocorticoids. Dexamethasone, spironolactone and eplerenone have been used in treatment.
DID YOU KNOW?
Although initially considered as a rare condition, the Glucocorticoid-remediable Aldosteronism is now regarded as one of the common causes of secondary hypertension in adults.