Autosomal dominant polycystic kidney disease is the most frequent life-threatening hereditary disease.
Prognostic factors for progressive renal impairment have been identified such as gender, race, age, proteinuria, hematuria,
hypertension. Hypertension is the only risk factor for renal dysfunction in autosomal dominant polycystic kidney disease,
which is presently treatable. Better understanding of the pathophysiology of hypertension will help in defining appropriate
interventions. The renin-angiotensin-aldosterone-system is the pivotal factor in the pathogenesis of hypertension in
autosomal dominant polycystic kidney disease. Basic research and clinical studies in autosomal dominant polycystic
kidney disease implicated activation of the renin-angiotensin-aldosterone-system. Therapy of hypertension in autosomal
dominant polycystic kidney disease with angiotensin-converting enzyme inhibitors or angiotensin receptor blocker has the
potential to prevent cardiovascular complications and slow the progression of renal disease. The results of two large
multicenter double-blind placebo controlled randomized clinical trials (the HALT-PKD trials) possibly will elucidate the
beneficial effects of the renin-angiotensin-aldosterone-system inhibition in autosomal dominant polycystic kidney disease.