IntroductionEndocrine forms of secondary hypertension (HTN) have traditionally included syndromes of mineralocorticoid excess with primary aldosteronism (PA) representing the most common etiology, followed by Cushing's syndrome (CS), and pheochromocytoma. Besides these conditions of hormonal excess, there are also other rare conditions such as congenital adrenal hyperplasia (11β-hydroxylase, 17α-hydroxylase deficiency), apparent mineralocorticoid excess, Geller syndrome (mineralocorticoid receptor (MR) activation), Liddle syndrome, pseudohypoaldosteronism type 2, and Chrousos syndrome that can cause HTN [1-4] (see Fig. 16.1).When considering the current obesity epidemic, with its many hormonal derangements (e.g., deficiencies of testosterone, vitamin D, growth hormone), the hitherto estimated prevalence rates of approximately 10 % for endocrine HTN are likely to be an underestimate. Potential nonadrenal causes of endocrine HTN include excess production of growth hormone (acromegaly), thyroid hormone, and parathyroid hormone, as well as insulin resistance, hypothyroidism, and overstimulation of central MRs [1,. These "nontraditional" forms of endocrine HTN will not be discussed in detail in this chapter.Cutoffs of systolic and diastolic blood pressure (BP) for defining HTN along with reference ranges for hormonal assays among various patient populations are 186 C. A. Koch et al. all important to consider when applying existing or emerging data to clinical patient scenarios. HTN guidelines by various societies have been recently revisited [41][42][43][44][45][46]. The American Society of Hypertension/International Society of Hypertension (ASH/ISH) guidelines reaffirm the traditional threshold of 140/90 mmHg as a cutoff for defining an elevated BP and state that individuals with a BP of 140-159/90-99 mmHg and no other risk factors are considered at low risk. On the other hand, the Joint National Committee (JNC 8) suggests a systolic blood pressure (SBP) of 150 mmHg as a worrisome threshold, especially for those 80 years and older. For the diastolic BP goal, both the ASH/ISH and JNC 8 guidelines view 90 mmHg as the cutoff; the exception are diabetic hypertensives for whom the cutoff is 85 mmHg. From epidemiologic studies and drug trials, there appears no sound evidence to continue recommending a BP of 140/90 mmHg as the cutoff to initiate antihypertensive treatment; moreover, any increased benefit from additional antihypertensive medications can be nonexistent or negligible, particularly in the face of increased likelihood of adverse effects. .1 Inherited endocrine condition related to mineralocorticoid excess. The picture shows the molecular pathways involved in dysregulation of NaCl homeostasis located in the distalrenal tubules. AME apparent mineralocorticoid excess; GRA glucocorticoid-remediable aldosteronism; PHA2 pseudohypoaldosteronism type 2; MR mineralocorticoid receptor; WNK with-no-lysine ( K) kinase 1,4; ROMK renal outer medullary potassium channel; ENaC epithelial sodium channel; KLHL3 kelch-like 3; C...