Abstract

BackgroundLittle is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). Recent studies suggest that QoL in ADPKD is determined by more factors than mere renal function. We investigated the effect of ADPKD on QoL and evaluated how Qol is affected by disease severity markers renal function, kidney volume and liver volume.MethodsWe performed a systematic review, meta-analysis and meta-regression analyses of cohort studies and randomized controlled trials investigating patient-reported QoL in adult patients with ADPKD not yet on dialysis. EMBASE, MEDLINE, and Web of Science were searched to August 2015 without language restrictions. Two investigators independently reviewed title, abstracts and full text of potentially relevant citations to determine eligibility. We compared pooled QoL summary scores of ADPKD patients using a random-effects meta-analytic model. These scores were compared with mean and age-corrected reference scores of the general population. In a meta-regression analysis, we investigated the univariate effect of renal function, kidney volume and liver volume on QoL.ResultsWe included nine studies in meta-analysis including 1623 patients who completed the SF-36 questionnaire. Pooled physical (PCS) and mental component scores (MCS) of the SF-36 of individuals with ADPKD were lower than those of the reference population (45.7 vs. 50.0 and 47.8 vs. 50.0 points, both P < 0.001). QoL of ADPKD patients remained lower after comparison with age-corrected reference values (age 35–44 year; PCS 52.2, MCS 49.9 points, both P < 0.05). Larger liver volume negatively impacted PCS (P < 0.001) and MCS (P = 0.001), whereas there was no association with renal function (PCS P = 0.1, MCS P = 0.9) and kidney volume (PCS P = 0.5, MCS P = 0. 5). Total liver and kidney volume had no impact on PCS (P = 0.1), but did have impact on MCS (P = 0.02).ConclusionsQoL reported by non-dialysis patients with ADPKD is impaired compared to the general population. Large liver volume was the most important factor that diminishes QoL.PROSPERO International Registry number CRD42015026428.

Highlights

  • Little is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD)

  • QoL reported by non-dialysis patients with ADPKD is impaired compared to the general population

  • As there is no validated total score of the gastrointestinal symptoms scale and the EQ-5D was used in only two studies, this resulted in exclusion of two studies for meta-analysis [40, 41]

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Summary

Introduction

Little is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). A growing body of evidence on QoL in chronic kidney disease (CKD) suggests that quality of life (QoL) is not determined merely by renal function [1,2,3]. In autosomal dominant polycystic kidney disease (ADPKD), anemia [4, 5], and cardiovascular diseases [6] are less frequent compared to other kidney diseases. This suggests that other disease-specific factors may contribute to QoL impairment in ADPKD [7]. Clinical symptoms in ADPKD seems to be a function of kidney and liver size, and are not primarily related to kidney function decline [11]

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