Treatment of autosomal dominant polycystic kidney disease

Abstract:

Autosomal dominant polycystic kidney disease (ADPKB) is the cause of end-stage chronic kidney disease (ESKD) in a significant number of patients on renal replacement therapy (NBF) worldwide and in Croatia. Despite the great advances in medicine during the past century, no therapeutic option to prevent cysts formation in those patients has been found so far. Treatment of ADPKD consists of dietetic measures, pharmacotherapy and RRT. Reduced intake of salt, increased fluids intake and blood pressure lowering by renin-angiotensin system inhibitors have been the treatment of choice for those patients for already long time. The outcomes of treatment should be a slowing of cysts growth and consecutive kidney enlargement and delay of ESKD onset. Currently, specific pharmacotherapy has been limited to vasopressin V2 receptors antagonists like tolvaptan, since recently. Patients at risk for rapid progression towards ESKD are candidates for such treatment. Based on previous studies, it can be approximated that tolvaptan can be used to prolong the progression to the final stage of CBD from 6 to 9 years, with an initial glomerular filtration rate of less than 60 mL/ min/1.73 m2 of body surface area. In conclusion, tolvaptan presents, at least to a certain extent, a hope for more effective treatment of ADPKD. However, antihypertensives and other nonspecific measures remain the foundations of ADPKD therapy and RRT remains the greatest achievement in the treatment of ADPKD patients.

Key words:
autosomal dominant polycystic kidney disease; tolvaptan; treatment; V2 vasopressin receptor antagonists