Autosomal-dominant Polycystic Kidney Disease (ADPKD): Executive Summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

Overview

Journal Kidney Int

Publisher Elsevier

Specialty Nephrology

Date 2015 Mar 19

PMID 25786098

Citations 249

Authors

Arlene B Chapman

Olivier Devuyst

Kai-Uwe Eckardt

Ron T Gansevoort

Tess Harris

Shigeo Horie

Bertram L Kasiske

Dwight Odland

York Pei

Ronald D Perrone

Yves Pirson

Robert W Schrier

Roser Torra

Vicente E Torres

Terry Watnick

David C Wheeler

Affiliations

Soon will be listed here.

Abstract

Autosomal-dominant polycystic kidney disease (ADPKD) affects up to 12 million individuals and is the fourth most common cause for renal replacement therapy worldwide. There have been many recent advances in the understanding of its molecular genetics and biology, and in the diagnosis and management of its manifestations. Yet, diagnosis, evaluation, prevention, and treatment vary widely and there are no broadly accepted practice guidelines. Barriers to translation of basic science breakthroughs to clinical care exist, with considerable heterogeneity across countries. The Kidney Disease: Improving Global Outcomes Controversies Conference on ADPKD brought together a panel of multidisciplinary clinical expertise and engaged patients to identify areas of consensus, gaps in knowledge, and research and health-care priorities related to diagnosis; monitoring of kidney disease progression; management of hypertension, renal function decline and complications; end-stage renal disease; extrarenal complications; and practical integrated patient support. These are summarized in this review.

Citing Articles

Digital physical activity intervention via the Kidney BEAM platform in patients with polycystic kidney disease: a randomized controlled trial.

Briggs J, Ralston E, Wilkinson T, Walklin C, Mangahis E, Young H Clin Kidney J. 2025; 18(3):sfaf041.

PMID: 40065847 PMC: 11892433. DOI: 10.1093/ckj/sfaf041.

A Challenging Case of Inferior Vena Cava Compression in an Adult Polycystic Kidney Disease Patient. A Case Report.

Matarneh A, Akkari A, Sardar S, Miller R, Verma N, Ghahramani N Clin Case Rep. 2025; 13(3):e70283.

PMID: 40034720 PMC: 11873366. DOI: 10.1002/ccr3.70283.

The impact of asymptomatic kidney stones on disease progression in autosomal dominant polycystic kidney disease.

Elcioglu O, Yatci B, Ozturk B, Mirioglu S, Gursu M, Kazancioglu R BMC Nephrol. 2025; 26(1):94.

PMID: 39994555 PMC: 11853763. DOI: 10.1186/s12882-025-03979-7.

Novel PKD1 Mutation (c.G10086T) Drives High Intracranial Aneurysm Risk in Autosomal Dominant Polycystic Kidney Disease.

Gao L, Lin M, Wu C, Liao Y, Lin Z, Yan X Eur J Neurol. 2025; 32(2):e70086.

PMID: 39973757 PMC: 11840425. DOI: 10.1111/ene.70086.

Multisystemic impact of autosomal dominant polycystic kidney disease: A case report highlighting renal, hepatic, and neurological involvement.

Khalil I, Faiyaz A, Hossain M, Akter M Radiol Case Rep. 2025; 20(4):2093-2100.

PMID: 39944160 PMC: 11821360. DOI: 10.1016/j.radcr.2025.01.021.

References

Devuyst O, Knoers N, Remuzzi G, Schaefer F . Rare inherited kidney diseases: challenges, opportunities, and perspectives. Lancet. 2014; 383(9931):1844-59. PMC: 4135047. DOI: 10.1016/S0140-6736(14)60659-0. View

Torres V, Harris P, Pirson Y . Autosomal dominant polycystic kidney disease. Lancet. 2007; 369(9569):1287-1301. DOI: 10.1016/S0140-6736(07)60601-1. View

Qu M, Ramirez-Giraldo J, Leng S, Williams J, Vrtiska T, Lieske J . Dual-energy dual-source CT with additional spectral filtration can improve the differentiation of non-uric acid renal stones: an ex vivo phantom study. AJR Am J Roentgenol. 2011; 196(6):1279-87. PMC: 3901037. DOI: 10.2214/AJR.10.5041. View

Chang L, Huang C, Tsai Y, Hung C, Fang M, Lin Y . Blastocyst biopsy and vitrification are effective for preimplantation genetic diagnosis of monogenic diseases. Hum Reprod. 2013; 28(5):1435-44. DOI: 10.1093/humrep/det048. View

Vikse B . Pre-eclampsia and the risk of kidney disease. Lancet. 2013; 382(9887):104-6. DOI: 10.1016/S0140-6736(13)60741-2. View

Fuller T, Brennan T, Feng S, Kang S, Stock P, Freise C . End stage polycystic kidney disease: indications and timing of native nephrectomy relative to kidney transplantation. J Urol. 2005; 174(6):2284-8. DOI: 10.1097/01.ju.0000181208.06507.aa. View

Haseebuddin M, Tanagho Y, Millar M, Roytman T, Chen C, Clayman R . Long-term impact of laparoscopic cyst decortication on renal function, hypertension and pain control in patients with autosomal dominant polycystic kidney disease. J Urol. 2012; 188(4):1239-44. DOI: 10.1016/j.juro.2012.06.026. View

ONeill W, Robbin M, Bae K, Grantham J, Chapman A, Guay-Woodford L . Sonographic assessment of the severity and progression of autosomal dominant polycystic kidney disease: the Consortium of Renal Imaging Studies in Polycystic Kidney Disease (CRISP). Am J Kidney Dis. 2005; 46(6):1058-64. DOI: 10.1053/j.ajkd.2005.08.026. View

Jiang T, Wang P, Qian Y, Zheng X, Xiao L, Yu S . A follow-up study of autosomal dominant polycystic kidney disease with intracranial aneurysms using 3.0 T three-dimensional time-of-flight magnetic resonance angiography. Eur J Radiol. 2013; 82(11):1840-5. DOI: 10.1016/j.ejrad.2013.01.024. View

10.

Sallee M, Rafat C, Zahar J, Paulmier B, Grunfeld J, Knebelmann B . Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2009; 4(7):1183-9. PMC: 2709515. DOI: 10.2215/CJN.01870309. View

11.

Meijer E, Drenth J, dAgnolo H, Casteleijn N, De Fijter J, Gevers T . Rationale and design of the DIPAK 1 study: a randomized controlled clinical trial assessing the efficacy of lanreotide to Halt disease progression in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2013; 63(3):446-55. PMC: 4042404. DOI: 10.1053/j.ajkd.2013.10.011. View

12.

Mufti U, Nalagatla S . Nephrolithiasis in autosomal dominant polycystic kidney disease. J Endourol. 2010; 24(10):1557-61. DOI: 10.1089/end.2010.0093. View

13.

Pei Y, Obaji J, Dupuis A, Paterson A, Magistroni R, Dicks E . Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2008; 20(1):205-12. PMC: 2615723. DOI: 10.1681/ASN.2008050507. View

14.

Orskov B, Sorensen V, Feldt-Rasmussen B, Strandgaard S . Improved prognosis in patients with autosomal dominant polycystic kidney disease in Denmark. Clin J Am Soc Nephrol. 2010; 5(11):2034-9. PMC: 3001783. DOI: 10.2215/CJN.01460210. View

15.

van Keimpema L, Nevens F, Adam R, Porte R, Fikatas P, Becker T . Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study. Transpl Int. 2011; 24(12):1239-45. DOI: 10.1111/j.1432-2277.2011.01360.x. View

16.

Bakker J, Olree M, Kaatee R, de Lange E, Moons K, Beutler J . Renal volume measurements: accuracy and repeatability of US compared with that of MR imaging. Radiology. 1999; 211(3):623-8. DOI: 10.1148/radiology.211.3.r99jn19623. View

17.

Jacquet A, Pallet N, Kessler M, Hourmant M, Garrigue V, Rostaing L . Outcomes of renal transplantation in patients with autosomal dominant polycystic kidney disease: a nationwide longitudinal study. Transpl Int. 2011; 24(6):582-7. DOI: 10.1111/j.1432-2277.2011.01237.x. View

18.

Kumar S, Fan S, Raftery M, Yaqoob M . Long term outcome of patients with autosomal dominant polycystic kidney diseases receiving peritoneal dialysis. Kidney Int. 2008; 74(7):946-51. DOI: 10.1038/ki.2008.352. View

19.

Patel P, Horsfield C, Compton F, Taylor J, Koffman G, Olsburgh J . Native nephrectomy in transplant patients with autosomal dominant polycystic kidney disease. Ann R Coll Surg Engl. 2011; 93(5):391-5. PMC: 3365458. DOI: 10.1308/003588411X582690. View

20.

Collins S . Preimplantation genetic diagnosis: technical advances and expanding applications. Curr Opin Obstet Gynecol. 2013; 25(3):201-6. DOI: 10.1097/GCO.0b013e32835faafe. View