Health-related quality of life in people with autosomal dominant polycystic kidney disease: a systematic review

Background

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited kidney disorder and a major contributor to kidney failure worldwide. However, the impact of ADPKD on health-related quality of life (HRQoL) across chronic kidney disease (CKD) stages and kidney replacement therapies (KRT) is poorly understood. This study aimed to synthesise existing evidence on HRQOL as measured by Patient-Reported Outcome Measures (PROMs) in people with ADPKD, stratified by disease stage and KRT modality.

Methods

A systematic review was conducted using five databases (Medline, Embase, PsycINFO, CINAHL, Web of Science) and Google Scholar to identify studies published between January 2014 and October 2024. Eligible studies reported HRQoL in individuals with ADPKD using generic, kidney-specific, or ADPKD-specific PROMs. Study populations were stratified by CKD stage and KRT modality. Scores were adjusted using country-specific population norms matched for age and sex, with population multipliers calculated to express Patient Reported Outcomes (PROs) as a proportion of the reference population.

Results

Six studies assessed PROs using the Short Form-36/12 survey. Physical health worsened with CKD progression, corresponding to lower values relative to matched population norms. Mental health showed smaller deviations from population norms. Dialysis patients had the lowest physical health multipliers, while transplant recipients had better physical health it did not improve to early-stage CKD levels. Two studies using the EuroQual 5-Dimension tool had less notable differences between CKD stages. Kidney disease and ADPKD-specific scores showed more pronounced declines across CKD stages than generic PROMs, suggesting greater sensitivity to stage-related changes.

Conclusions

This review demonstrates that PROs for individuals with ADPKD are lower in later CKD stages compared to earlier stages, with the largest effect on physical health. Mental health scores were less affected suggesting adaptation over time. Our findings suggest generic PROMs may underestimate the impact of ADPKD compared to disease-specific tools.