Management of Rapidly Progressive Autosomal Dominant Polycystic Kidney Disease

Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease. In this disease, multiple cysts develop and grow over time in both kidneys. As a result, kidney function decreases over the years, patients develop end stage renal disease, and they need kidney replacement therapy. The search for drugs that can slow the growth of cysts in ADPKD has been going on for many years. Experimental and clinical studies conducted to date have demonstrated that , “tolvaptan,” a vasopressin receptor antagonist has beneficial effects. There is no specific treatment other than tolvaptan currently used worldwide for rapidly progressive polycystic kidney disease. However, physicians experience difficulties in various processes with tolvaptan, such as identifying the appropriate patient group, planning the treatment process, determining the ideal dosage, and managing complications. For this reason, we decided to prepare a practical treatment guide to help supporting clinicians in the management of ADPKD patients in clinical practice.

Cite this article as: Koçyiğit İ, Aydın E, Oto ÖA, Atılgan KG, Ecder T. Management of rapidly progressive autosomal dominant polycystic kidney disease. Turk J Nephrol. Published online September 1, 2025.