Consensus document on autosomal dominant polycystic kindey disease from the Spanish Working Group on Inherited Kindey Diseases. Review 2020

Ars, Elisabet; Bernis, Carmen; Fraga, Gloria; Furlano, Mónica; Martínez, Víctor; Martins, Judith; Ortiz, Alberto; Pérez-Gómez, Maria Vanessa; Rodríguez-Pérez, José Carlos; Sans Atxer, Laia; Torra, Roser

doi:http://dx.doi.org/10.1016/j.nefroe.2022.11.011

Consensus document on autosomal dominant polycystic kindey disease from the Spanish Working Group on Inherited Kindey Diseases. Review 2020

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Ars E, Bernis C, Fraga G, Furlano M, Martínez V, Martins J, Ortiz A, Pérez-Gómez MV, Rodríguez-Pérez JC, Sans L, Torra R; en nombre del grupo de trabajo de Enfermedades Renales Hereditarias de la Sociedad Española de Nefrología. Consensus document on autosomal dominant polycystic kindey disease from the Spanish Working Group on Inherited Kindey Diseases. Review 2020. Nefrologia (Engl Ed). 2022 Jul-Aug;42(4):367-89. DOI: 10.1016/j.nefroe.2022.11.011

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent cause of genetic renal disease and accounts for 6-10% of patients on kidney replacement therapy (KRT). Very few prospective, randomized trials or clinical studies address the diagnosis and management of this relatively frequent disorder. No clinical guidelines are available to date. This is a revised consensus statement from the previous 2014 version, presenting the recommendations of the Spanish Working Group on Inherited Kidney Diseases, which were agreed to following a literature search and discussions. Levels of evidence mostly are C and D according to the Centre for Evidence-Based Medicine (University of Oxford). The recommendations relate to, among other topics, the use of imaging and genetic diagnosis, management of hypertension, pain, cyst infections and bleeding, extra-renal involvement including polycystic liver disease and cranial aneurysms, management of chronic kidney disease (CKD) and KRT and management of children with ADPKD. Recommendations on specific ADPKD therapies are provided as well as the recommendation to assess rapid progression.