原発性アルドステロン症

Primary aldosteronism (PA) results from excessive aldosterone production by one or both adrenal glands and is an important cause of hypertension, leading to increased cardiovascular and renal morbidities. PA is primarily caused by a spectrum of somatic or germline mutations in aldosterone-driver genes and superimposed aberrant adrenal expression of various G-protein-coupled receptors and their ligands, leading to dysregulated aldosterone production. PA remains underdiagnosed, and simplified testing by measuring renin and aldosterone is recommended in all people with hypertension to maximize the diagnosis of PA. Some individuals with PA may also have co-secretion of cortisol, which contributes to cardiometabolic morbidities. Adrenal vein sampling, emerging functional imaging and novel biomarkers can identify whether a unilateral source of PA can be treated with surgical adrenalectomy. However, the majority of patients with PA have bilateral disease, warranting medical therapy with dietary sodium restriction and mineralocorticoid receptor antagonists, and aldosterone synthase inhibitors in the near future. Medical therapy objectives are to normalize blood pressure and serum potassium; a rise in renin can serve as a biomarker of adequate therapy and reduced risk for adverse cardio-renal outcomes. Patients with PA should be monitored longitudinally for disease progression or recurrence, to manage potential adverse effects of treatment, and to optimize therapy of cardiovascular and other co-morbidities.