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Diagnosis and Management of Primary Aldosteronism

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Diagnosis and Management of Primary Aldosteronism 1 179 T A Williams and M Reincke Management of primary 179:1 R19–R29 Review aldosteronism MANAGEMENT OF ENDOCRINE DISEASE Diagnosis and management of primary aldosteronism: the Endocrine Society guideline 2016 revisited Tracy Ann Williams1,2 and Martin Reincke1 Correspondence 1 Medizinische Klinik und Poliklinik IV, Klinikum der Universität, Ludwig-Maximilians-Universität München, Munich, should be addressed 2 Germany and Division of Internal Medicine and Hypertension, Department of Medical Sciences, University of Turin, to M Reincke Turin, Italy Email Martin.Reincke@med. uni-muenchen.de Abstract The syndrome of primary aldosteronism (PA) is characterized by hypertension with excessive, autonomous aldosterone production and is usually caused by either a unilateral aldosterone-producing adenoma or bilateral adrenal hyperplasia. The diagnostic workup of PA is a sequence of three phases comprising screening tests, confirmatory tests and the differentiation of unilateral from bilateral forms. The latter step is necessary to determine the optimal treatment approach of unilateral laparoscopic adrenalectomy (for patients with unilateral PA) or medical treatment with a mineralocorticoid receptor antagonist (for patients with bilateral PA). Since the publication of the revised Endocrine Society guideline 2016, a number of key studies have been published. They challenge the recommendations of the guideline in some areas and confirm current practice in others. Herein, we present the recent developments and current approaches to the medical management of PA. European Journal European of Endocrinology European Journal of Endocrinology (2018) 179, R19–R29 Introduction Primary aldosteronism (PA), first described by Jerome W. renin (1). The application of the current screening Conn in 1955, was once thought to be a rare condition method of an elevated plasma aldosterone-to-renin ratio of hypertension characterized by hypokalemia and excess (ARR) to a wider target population (instead of restricted to aldosterone production relative to suppressed plasma patients with hypokalemia) accounting for up to 50% of Invited Author’s profile Prof. Dr med. Martin Reincke is the Director of the Medizinische Klinik and Poliklinik IV, Ludwig-Maximilians University in Munich and Chair of the Department of Endocrinology and Diabetology. His research focus includes adrenal physiology and pathophysiology, endocrine hypertension, pituitary disease, mineralocorticoid and glucocorticoid action and stress research. Prof. Reincke is heading a research team specifically exploring the prevalence and relevance of mineralocorticoid excess in resistant hypertension on the epidemiological, clinical, genetic and molecular levels. www.eje-online.org © 2018 European Society of Endocrinology Published by Bioscientifica Ltd. https://doi.org/10.1530/EJE-17-0990 Printed in Great Britain Downloaded from Bioscientifica.com at 09/27/2021 05:58:35AM via free access 10.1530/EJE-17-0990 Review T A Williams and M Reincke Management of primary 179:1 R20 aldosteronism the population with hypertension has greatly increased the diagnosis of PA (2). It is now widely accepted that this syndrome is the most common form of endocrine hypertension. PA is specifically treated by unilateral adrenalectomy (preferably by laparoscopic surgery) or pharmacologically with a mineralocorticoid receptor (MR) antagonist that competitively inhibits the binding of aldosterone to the MR (3). Patients with PA have an increased risk of cardiovascular and cerebrovascular events and target organ damage (heart and kidney) relative to patients with essential hypertension and a matched cardiovascular risk profile (4, 5, 6, 7) or compared with the general population with hypertension (8). Patients with PA also display an increased prevalence of metabolic syndrome and diabetes (4, 9, 10, 11), osteoporotic fractures (12) and symptoms of depression with a reduced quality of life (13, 14). Some of these comorbidities may be associated with cortisol co-secretion (15). All available evidence indicates that an early diagnosis and appropriate clinical management (surgical or medical) is mandatory to minimize the increased risks associated with PA (5, 16, 17, 18). The diagnostic management of PA comprises three phases: screening tests, case confirmation and differentiation of unilateral from bilateral forms of PA for therapeutic decision making and appropriate treatment (Fig. 1). Figure 1 Flow chart for the diagnostic workup of primary European Journal European of Endocrinology aldosteronism. *Please refer to Table 1 for patients with Diagnosis: screening hypertension to be screened for PA ADX, adrenalectomy; CT, Measurement of plasma aldosterone concentrations computer-assisted tomography; MR, mineralocorticoid (PACs) and plasma renin activity (PRA) or the direct receptor; PA, primary aldosteronism. renin concentration (DRC) to assess the ARR is the most reliable currently available method of screening for PA. system, and specifically those that may stimulate renin The Endocrine Society (ES) Clinical Practice Guideline secretion, should be withdrawn (this applies throughout recommends screening patients with an increased the diagnostic workup for PA because other tests and likelihood of PA (Table 1) (3). It has been suggested that procedures also rely on measurements of steroids under all patients with hypertension should be screened for conditions of suppressed renin). Antihypertensive PA (19), based on the findings of the prospective PATO medication that interferes with the ARR includes diuretics study (8), which reported a 5.9% prevalence of PA in (including spironolactone) and should be withdrawn for 1672 unselected patients with hypertension in primary 4 weeks. Other drugs that should be withdrawn, but for care (8). However, evidence that a systematic screening a lesser period of 2 weeks prior to testing, are β-blockers, approach (compared with selective screening) results in clonidine, methyldopa, non-steroidal anti-inflammatory a reduction of morbidity, mortality and cardiovascular drugs, ACE inhibitors, angiotensin receptor blockers and disease of patients with hypertension to an extent that dihydropyridine calcium blockers (3, 20). However, in would justify the increased costs and burden on health many instances, interpretation of the ARR is confidently systems is lacking. possible without changing interfering medications. Non- To screen for PA by the ARR, it is recommended dihydropyridine long-acting calcium channel blockers that medications interfering with the renin-angiotensin (verapamil or diltiazem), the vasodilator hydralazine www.eje-online.org Downloaded from Bioscientifica.com at 09/27/2021 05:58:35AM via free access Review T A Williams and M Reincke Management of primary 179:1 R21 aldosteronism Table 1 Screening for primary aldosteronism. An elevated ARR can result if the PRA (or DRC) is very low even if the PAC is low-normal and inconsistent Risk groups recommended to be screened for primary aldosteronism according to ES guideline with PA. Some centers do not proceed with the diagnostic Patients with sustained blood pressure above workup of PA of such patients and require a minimum 150/100 mmHg, grade 2 and grade 3 hypertension PAC in addition to an elevated ARR for a positive screening Patients with resistant hypertension (blood pressure not controlled by three conventional drugs including a test. At least, baseline PAC should be higher than the diuretic) or controlled BP (<140/90 mmHg) on four or normal range of confirmatory tests, as discussed later (20). more antihypertensive drugs Patients with hypertension and spontaneous or diuretic induced hypokalemia Patients with hypertension and an adrenal incidentaloma Diagnosis: confirmatory testing Patients with hypertension and sleep apnea Patients with hypertension and a family history of Confirmatory testing is considered mandatory by the ES early-onset hypertension or cerebrovascular accident at a young age (<40 years) guideline for a definitive diagnosis of PA. An exception is All first-degree relatives of patients with PA in evident cases of PA with spontaneous hypokalemia and a PAC > 20 ng/dL (550 pmol/L) with PRA (or DRC) below Includes data from Funder et al. (3) and Stowasser & Gordon (20). assay detection limits (3). Confirmation or exclusion of and α1-adrenergic receptor blockers have limited or the diagnosis of PA is performed by ≥1 confirmatory tests no effects on the ARR compared to the above and are as recommended by the ES guideline (3). suggested to control hypertension in those patients with Confirmatory tests demonstrate the inappropriate elevated blood pressure requiring medical treatment (3, production of aldosterone in response to exogenously 20). As a caveat, severe deleterious side effects have been administered agents that normally completely suppress or reported by adjustment of antihypertensive therapy in inhibit circulating angiotensin II levels (the endogenous accordance with the ES guideline during screening for positive regulator of aldosterone production). The PA (21). In our center, more than 90% of patients receive test thereby confirms that aldosterone production is adjusted medication according to the ES guideline autonomous of the renin-angiotensin system. during screening, and serious adverse events have been Potassium is a key regulator of aldosterone production reduced to a minimum after appropriate exclusion of and hypokalemia (if present) should be corrected with high-risk patients. slow-release KCl tablets
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