Treatment of Autosomal Dominant Polycystic Kidney Disease

ABSTRACT: Autosomal dominant polycystic kidney disease (ADPKD) is a systemic, hereditary disorder requiring lifelong, multidisciplinary management. Approximately half of affected individuals progress to kidney failure by age 60. Recent advances in genetics, imaging, and disease pathophysiology have enabled earlier diagnosis, improved risk stratification, and led to the development of disease-modifying therapies (such as tolvaptan) for high-risk patients. Despite the availability of comprehensive clinical practice guidelines, real-world implementation is hindered by disease heterogeneity, psychosocial impacts, and the need for individualized care. The aim of this narrative review is to facilitate guideline implementation by integrating evidence from patient perspectives, real-world effectiveness, and offering strategies to overcome practical barriers.

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Publication Details

Journal
Journal of the American Society of Nephrology
Published
2026-04-02
DOI
https://doi.org/10.1681/asn.0000001113
Primary Topic
Genetic and Kidney Cyst Diseases
Type
article
Field-Weighted Citation Impact
0.00
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article

Treatment of Autosomal Dominant Polycystic Kidney Disease

Gopala K. Rangan
Journal of the American Society of Nephrology
Genetic and Kidney Cyst Diseases
article

Treatment of Autosomal Dominant Polycystic Kidney Disease

Gopala K. Rangan
article en

Abstract

ABSTRACT: Autosomal dominant polycystic kidney disease (ADPKD) is a systemic, hereditary disorder requiring lifelong, multidisciplinary management. Approximately half of affected individuals progress to kidney failure by age 60. Recent advances in genetics, imaging, and disease pathophysiology have enabled earlier diagnosis, improved risk stratification, and led to the development of disease-modifying therapies (such as tolvaptan) for high-risk patients. Despite the availability of comprehensive clinical practice guidelines, real-world implementation is hindered by disease heterogeneity, psychosocial impacts, and the need for individualized care. The aim of this narrative review is to facilitate guideline implementation by integrating evidence from patient perspectives, real-world effectiveness, and offering strategies to overcome practical barriers.

Journal of the American Society of Nephrology
The University of Sydney (AU), Westmead Hospital (AU), Sydney Local Health District (AU), Westmead Institute for Medical Research (AU)
Openalex Percentile: Top 24%
Genetic and Kidney Cyst Diseases
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