Causes of Mineralocorticoid Excess

Causes of Mineralocorticoid Excess Mechanism % Primary Hyperaldosteronism  Adrenal (Conn's) adenoma Autonomous aldosterone excess 40 Bilateral (micronodular) adrenal hyperplasia Autonomous aldosterone excess 60 Glucocorticoid-remediable hyperaldosteronism (dexamethasone-suppressible hyperaldosteronism) Crossover between the CYP11B1 and CYP11B2 genes results in ACTH-driven aldosterone production <1 Other Causes (Rare)  <1 Syndrome of apparent mineralocorticoid excess (AME) Mutations in HSD11B2 result in lack of renal activation of cortisol to cortisone, leading to excess activation of the MR by cortisol   Cushing's syndrome Cortisol excess overcomes the capacity of HSD11B2 to inactivate cortisol to cortisone, consequently flooding the MR   Glucocorticoid resistance Upregulation of cortisol production due to GR mutations results in flooding of the MR by cortisol   Adrenocortical carcinoma Autonomous aldosterone and/or DOC excess   Congenital adrenal hyperplasia Accumulation of DOC due to mutations in CYP11B1 orCYP17A1    Progesterone-induced hypertension Progesterone acts as an abnormal ligand due to mutations in the MR gene   Liddle's syndrome Mutant ENaC beta or gamma subunits resulting in reduced degradation of ENaC keeping the membrane channel in open conformation for longer, enhancing mineralocorticoid action