Classic and Nonclassic Apparent Mineralocorticoid Excess Syndrome.

Cristian A Carvajal, Alejandra Tapia-Castillo, Andrea Vecchiola, Rene Baudrand, Carlos E Fardella

The Journal of clinical endocrinology and metabolism 2020 Apr 01

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Arterial hypertension (AHT) is one of the most frequent pathologies in the general population. Subtypes of essential hypertension characterized by low renin levels allowed the identification of 2 different clinical entities: aldosterone-mediated mineralocorticoid receptor (MR) activation and cortisol-mediated MR activation. This review is based upon a search of Pubmed and Google Scholar databases, up to August 2019, for all publications relating to endocrine hypertension, apparent mineralocorticoid excess (AME) and cortisol (F) to cortisone (E) metabolism. The spectrum of cortisol-mediated MR activation includes the classic AME syndrome to milder (nonclassic) forms of AME, the latter with a much higher prevalence (7.1%) than classic AME but different phenotype and genotype. Nonclassic AME (NC-AME) is mainly related to partial 11βHSD2 deficiency associated with genetic variations and epigenetic modifications (first hit) and potential additive actions of endogenous or exogenous inhibitors (ie, glycyrrhetinic acid-like factors [GALFS]) and other factors (ie, age, high sodium intake) (second hit). Subjects with NC-AME are characterized by a high F/E ratio, low E levels, normal to elevated blood pressure, low plasma renin and increased urinary potassium excretion. NC-AME condition should benefit from low-sodium and potassium diet recommendations and monotherapy with MR antagonists. NC-AME has a higher prevalence and a milder phenotypical spectrum than AME. NC-AME etiology is associated to a first hit (gene and epigene level) and an additive second hit. NC-AME subjects are candidates to be treated with MR antagonists aimed to improve blood pressure, end-organ damage, and modulate the renin levels. © Endocrine Society 2019. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Citation

Cristian A Carvajal, Alejandra Tapia-Castillo, Andrea Vecchiola, Rene Baudrand, Carlos E Fardella. Classic and Nonclassic Apparent Mineralocorticoid Excess Syndrome. The Journal of clinical endocrinology and metabolism. 2020 Apr 01;105(4)