Impact of liver volume on polycystic liver disease-related symptoms and quality of life

Abstract

Background: Symptoms in polycystic liver disease (PLD) are thought to be caused by compression of organs and structures by the enlarged liver.

Aim: The aim of this article is to assess the impact of liver volume on symptoms and quality of life (QoL) in PLD.

Methods: We included PLD patients from two prospective studies that used the PLD-questionnaire (PLD-Q) for symptom assessment. QoL was assessed through SF-36, summarized in a physical (PCS) and mental (MCS) component score. Liver volume was correlated with PLD-Q total scores. Patients were classified based on height-corrected liver volume in mild (<1600 ml), moderate (1600-3200 ml), and severe (>3200 ml) disease. PLD-Q and QoL (PCS and MCS) scores were compared across disease stages.

Results: We included 82 of 131 patients from the original studies (disease stages; mild n = 26, moderate n = 33, and severe n = 23). Patients with larger liver volume reported higher symptom burden (r = 0.516, p < 0.001). Symptom scores increased with disease progression, except for abdominal pain (p = 0.088). PCS decreased with advancing disease (p < 0.001), in contrast to MCS (p = 0.055). Moderate (p = 0.007) and severe (p < 0.001) PLD patients had lower PCS scores than the general population.

Conclusion: PLD with larger liver volume is more likely to be symptomatic and is associated with lower QoL.

Keywords: Autosomal dominant polycystic kidney disease (ADPKD); autosomal dominant polycystic liver disease (ADPLD); hepatomegaly; quality of life; symptoms.

Figure 1.

Flowchart shows reasons for exclusion from original studies. Of the 131 patients assessed for eligibility, 82 were included in the study. PLD-Q: polycystic liver disease questionnaire; DIPAK: Developing Interventions to halt Progression of autosomal dominant polycystic kidney disease (ADPKD); CURSOR: Controlled trial of URSOdeoxycholic acid to Reduce liver volume in polycystic liver disease

Figure 2.

Severity of individual symptoms (%) as scored on the polycystic liver disease questionnaire (PLD-Q) in the total study population. Scores are ranging from 2 to 11, whereas higher scores correspond with more severe symptoms (darker bars).

Figure 3.

Median individual symptom scores per disease stage. A severity score of 2-3 can be considered as no symptoms (center), 4-5 as mild, 6-7 as moderate, 8-9, as moderately-severe, and 10-11 as severe symptoms (most outer lines). PLD: polycystic liver disease; htLV: height-corrected liver volume.

Figure 4.

Left panel: physical component scores (PCS) decreased significantly with advancing disease stages (p = 0.021). Right panel: no significant effect of disease stage on the mental component scale (MCS) (p = 0.055). The dotted line reflects the reference value of the general population. Disease stages are defined as: mild <1600 mL, moderate 1600–3200 mL; and severe >3200 mL height corrected liver volume. PLD: polycystic liver disease; SF-36: Short-Form 36.