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Hypertension in autosomal dominant polycystic kidney disease

INTRODUCTION

Hypertension is common in most chronic progressive kidney diseases. However, the pathogenesis is somewhat different in autosomal dominant polycystic kidney disease (ADPKD).

Hypertension is a common early finding in ADPKD, occurring in 50 to 70 percent of cases before any significant reduction in glomerular filtration rate (GFR), with an average age onset of 30 years [1-3]. However, the tendency to develop hypertension and its complications begins even earlier (eg, left ventricular hypertrophy) [4]. Affected young adults have a higher ambulatory blood pressure and left ventricular mass index than age-matched controls, even though the values remain within the normal range [5,6]. This raises the possibility that treatment of normotensive ADPKD patients may be beneficial.

PATHOGENESIS

Increased activity of the renin-angiotensin system (RAS) and extracellular volume expansion are often present early in autosomal dominant polycystic kidney disease (ADPKD) (ie, prior to elevation in the serum creatinine) and may play an important role in the rise in blood pressure [7]. It has been suggested that cyst expansion, leading to focal areas of renal ischemia and enhanced renin release, is largely responsible for at least the initial rise in blood pressure [1,2]. Two observations are compatible with this hypothesis:

Renin-containing cells are present in the attenuated arteries in the walls of the cysts and in cells in the connective tissue surrounding cysts [8,9]. Renin can also be produced by the epithelial cells lining the cysts, and active renin is often present within the cyst fluid [9]. Renin may directly promote epithelial cell hyperplasia and cyst growth since angiotensin II is a growth factor.

The degree of early hypertension varies with the degree of structural change as patients with normal serum creatinine with an elevated blood pressure tend to have a higher total cyst volume than those who are normotensive [10].

      

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Literature review current through: Oct 2014. | This topic last updated: Jan 15, 2014.
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