Plasma copeptin is a marker of vasopressin and, in autosomal dominant polycystic kidney disease (ADPKD), might also indicate the extent of kidney damage. Patients with ADPKD (n = 122) had significantly higher mean plasma copetin levels than healthy kidney donors (n = 134), before and after donation (P <0.001). In these patients, but not in kidney donors, copeptin was significantly associated with glomerular filtration rate (P <0.001), implying that plasma copeptin is an ADPKD-specific indicator of renal function.