REN and ciliopathy: Hypertension occurs in 60% of patients before renal impairment in PKD, and is universal in chronic kidney disease stages 4 to 5.[19] Activation of the renin-angiotensin-aldosterone system, vascular dysfunction, related to ciliopathy and other factors have all been found to be involved in the development of hypertension in ADPKD.[20] Amelioration of hypertension was observed in case 2 after surgery without handling renal cysts.