The natural history of autosomal dominant polycystic kidney disease: 30-year experience from a single centre

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Background and Aim: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease. The major objective of this study was to analyse the natural history of disease progression in all patients with ADPKD seen at the Sheffield Kidney Institute between 1978 and 2012.

Methods: A retrospective analysis was performed based on recorded renal function up to 30 years prior to analysis. The rate of estimated glomerular filtration rate (eGFR) decline (ΔeGFR) was determined by linear regression, based on a minimum of 5 years of renal function prior to the study or renal replacement therapy. Patients who had reached end-stage renal disease (ESRD) (n = 113) were compared with those under follow-up in a dedicated polycystic kidney disease (PKD) clinic (n = 88).

Results: The two groups were comparable in age and gender though the mean follow-up duration was longer in the PKD clinic group. Overall, ΔeGFR was significantly higher in the ESRD group compared with the PKD clinic group (4.19 ± 1.66 vs. 1.71 ± 1.36 ml/min/1.73 m2/year). Retrospective analysis for each 5-year period prior to ESRD or analysis showed a significant difference in ΔeGFR between both groups 10 years before with an increasing trend in ΔeGFR 20 years before especially in the ESRD group. In the PKD clinic group, the age of diagnosis and mean kidney length were also predictors of ΔeGFR.

Conclusions: This is one of the longest natural history studies in ADPKD. The difference in ΔeGFR can be predicted at least 10 years prior to the onset of ESRD and thus will enable higher risk patients to be identified early for treatment.

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