REVIEWS Renal transplantation in autosomal dominant polycystic kidney disease Nada Kanaan, Olivier Devuyst and Yves Pirson Abstract | In patients with autosomal dominant polycystic kidney disease (ADPKD) evaluated for kidney transplantation, issues related to native nephrectomy, cystic liver involvement, screening for intracranial aneurysms and living-related kidney donation deserve special consideration. Prophylactic native nephrectomy is restricted to patients with a history of cyst infection or recurrent haemorrhage or to those in whom space must be made to implant the graft. Patients with liver involvement require pretransplant imaging. Selection of patients for pretransplant screening of intracranial aneurysms should follow the general recommendations for patients with ADPKD. In living related-donor candidates aged 70% of patients; total kidney volume is the strongest predictor of the rate of decline in renal function.12 ADPKD is genetically hetero­geneous as mutations in two genes are responsible for the disorder: PKD1 on chromosome 16 (which encodes p ­ olycystin‑­1) and PKD2 on chromosome 4 (which encodes p ­ olycystin‑­2).13–17 These genes are causative in 85% and 15% of patients with ADPKD, respectively.1 Patients with ADPKD caused by mutations in PKD1 have more-severe renal disease, larger kidneys and faster progression to ESRD than those with ADPKD caused by mutations in PKD2 (median age at onset of ESRD 53.4 years versus 72.7 years, respectively).12,18,19 In addition, genetic and environmental m ­ odifying factors can influence the course of the disease.20–23 For patients with ADPKD who reach ESRD, kidney transplantation is the preferred type of renal replacement therapy.24–29 A European survey of 35,164 patients with ADPKD receiving renal replacement therapy (haemo­ dialysis, peritoneal dialysis or renal transplantation)

Correspondence to: Y.P. yves.pirson@ uclouvain.be

Competing interests The authors declare no competing interests.

showed an increase in use of kidney transplantation from 1991 to 2010.30 During this 19-year period, the prevalence of patients with ADPKD living with a first renal transplant increased from 22 to 55 per million population, and the relative contribution of kidney transplantation to overall renal replacement therapy in ADPKD increased from 43% to 59%.30 Here, we discuss the pretransplant evaluation and post-transplant outcomes of patients with ADPKD, focusing on specific issues associated with this disease. Recommendations for the pretransplant evaluation of liver involvement and screening for intracranial aneur­ ysms largely rely on what has been learnt from the posttransplant outcomes of these two complications; thus, we consider each in a single section in the pretransplant evaluation section, whereas the other complications are discussed separately in both the pretransplant and ­post-transplant sections of this Review.

Pretransplant evaluation Issues related to native nephrectomy, cystic liver involvement, screening for intracranial aneurysms and living related-donor transplantation are important considerations in patients with ADPKD who are being evaluated for kidney transplantation (Box 1, Figure 1).

Native nephrectomy Controversy exists concerning the need, indication, timing and approach for native nephrectomy in patients with ADPKD undergoing kidney transplantation.31,32

NATURE REVIEWS | NEPHROLOGY

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REVIEWS Key points ■■ In patients with autosomal dominant polycystic kidney disease (ADPKD), rates of patient and graft survival following kidney transplantation are excellent ■■ Prophylactic native nephrectomy is restricted to patients with severe pain, early satiety, recurrent bleeding, infections or stones and those in whom space must be made to implant the graft ■■ Patients with liver involvement require pretransplant imaging; combined liver–kidney transplantation should be considered in cases of symptomatic hepatomegaly or recurrent cholangitis if the glomerular filtration rate is ≤30 ml/min/1.73 m2 ■■ General recommendations for the screening of intracranial aneurysms in patients with ADPKD should be used to select patients for pretransplant screening ■■ Genetic testing should be done in living related-donor candidates aged