Introduction
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder, with a prevalence of 1:500 to 1:1000. 1 ADPKD accounts for 7% to 11% of patients on renal replacement therapy in Europe and approximately 5% of patients requiring dialysis in the United States. 2 ADPKD is characterized by bilateral renal cysts and may be associated with kidney pain, urinary tract infection, hematuria, nephrolithiasis, hypertension, and progressive renal failure due to progressive enlargement of cysts and fibrosis.3-5 Cyst growth displaces and destroys normal kidney tissue, culminating in fibrosis, renal architectural derangement, and ultimately kidney failure. 6,7 An estimated 45% to 70% of patients with ADPKD progress to end-stage renal disease (ESRD) by age 65 years. 8
Several targeted pharmacological therapies have been tested in patients with ADPKD, including mammalian target of rapamycin (mTOR) inhibitors, somatostatin analogues, and the vasopressin V2-receptor antagonist tolvaptan2 ; however, tolvaptan is the only approved therapy in Canada for the treatment of ADPKD.9 The purpose of this consensus recommendation is to develop an evidence-informed recommendation for the optimal management of adult patients with ADPKD. The focus will be on the role of genetic testing, the role of renal imaging, risk prediction of disease progression, and pharmacological treatment options. These areas of focus were derived from 2 national surveys that were disseminated to nephrologists and patients with ADPKD.