RESUMO
Autosomal dominant polycystic kidney disease is the most common genetic kidney disease affecting adults. Approximately 60% of patients develop kidney failure by 60 years of age due to slowly expanding kidney cysts. A healthy lifestyle and rigorous control of blood pressure slow kidney cyst growth. These interventions can be effective in reducing progression to kidney failure and cardiovascular disease, especially if started in early adulthood. Tolvaptan, a vasopressin receptor antagonist, slows kidney cyst growth and the decline in the estimated glomerular filtration rate by 1 mL/minute/1.73 m2 per year. It is indicated in patients with chronic kidney disease who are at high risk of progression to kidney failure. Chronic kidney pain is common and can be managed with analgesics, and input from pain specialists if refractory.