Vascular complications in autosomal dominant polycystic kidney disease

Ronald D. Perrone, Adel M. Malek, Terry Watnick

Research output: Contribution to journalArticle

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Relentless cyst growth substantially enlarges both kidneys and culminates in renal failure. Patients with ADPKD also have vascular abnormalities; intracranial aneurysms (IAs) are found in â 1/410% of asymptomatic patients during screening and in up to 25% of those with a family history of IA or subarachnoid haemorrhage. As the genes responsible for ADPKD - PKD1 and PKD2 - have complex integrative roles in mechanotransduction and intracellular calcium signalling, the molecular basis of IA formation might involve focal haemodynamic conditions exacerbated by hypertension and altered flow sensing. IA rupture results in substantial mortality, morbidity and poor long-term outcomes. In this Review, we focus mainly on strategies for screening, diagnosis and treatment of IAs in patients with ADPKD. Other vascular aneurysms and anomalies - including aneurysms of the aorta and coronary arteries, cervicocephalic and thoracic aortic dissections, aortic root dilatation and cerebral dolichoectasia - are less common in this population, and the available data are insufficient to recommend screening strategies. Treatment decisions should be made with expert consultation and be based on a risk-benefit analysis that takes into account aneurysm location and morphology as well as patient age and comorbidities.

Original languageEnglish (US)
Pages (from-to)589-598
Number of pages10
JournalNature Reviews Nephrology
Volume11
Issue number10
DOIs
StatePublished - Oct 22 2015
Externally publishedYes

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Autosomal Dominant Polycystic Kidney
Intracranial Aneurysm
Blood Vessels
Aneurysm
Inborn Genetic Diseases
Calcium Signaling
Intracranial Hemorrhages
Kidney Diseases
Subarachnoid Hemorrhage
Renal Insufficiency
Aorta
Dissection
Comorbidity
Cysts
Rupture
Dilatation
Coronary Vessels
Thorax
Referral and Consultation
Hemodynamics

ASJC Scopus subject areas

  • Nephrology

Cite this

Vascular complications in autosomal dominant polycystic kidney disease. / Perrone, Ronald D.; Malek, Adel M.; Watnick, Terry.

In: Nature Reviews Nephrology, Vol. 11, No. 10, 22.10.2015, p. 589-598.

Research output: Contribution to journalArticle

Perrone, Ronald D. ; Malek, Adel M. ; Watnick, Terry. / Vascular complications in autosomal dominant polycystic kidney disease. In: Nature Reviews Nephrology. 2015 ; Vol. 11, No. 10. pp. 589-598.
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