Quality of Life in Patients With Primary Sclerosing Cholangitis.

BACKGROUND AND AIMS: Primary sclerosing cholangitis (PSC) is an autoimmune chronic cholestatic liver disease causing inflammation, fibrosis, and increased risk of cholangitis, cirrhosis, and hepatobiliary cancers. How PSC affects patient-reported quality of life (QoL) is sparsely studied. We aimed to investigate the impact of PSC on QoL.

METHODS

This cross-sectional study included 101 patients with PSC. We investigated QoL using the PSC-specific PSC patient-reported outcome (PRO) instrument, the generic health-related QoL questionnaire EQ-5D-5L, and the fatigue visual analog scale (VAS) score. We also investigated associations between QoL and noninvasive inflammation and fibrosis measures; the macrophage marker soluble (s)CD163, fibrosis-4 score, and transient elastography.

RESULTS

The median age was 45 years, 65% were males, 10% had PSC with autoimmune hepatitis overlap disease, and 92% had inflammatory bowel disease. Eighty-five patients completed all QoL questionnaires and reported high QoL across all PSC PRO instrument items and EQ-5D-5L. sCD163 correlated modestly with PSC PRO total score (rho = 0.28) and fatigue VAS score (rho = 0.26) but not EQ-5D-5L. Patients with any previous episode of cholangitis reported lower QoL than cholangitis-naive patients on the PSC PRO score (21 vs 15, P = .04) and EQ-5D-5L VAS score (71 vs 86, P = .01). There was no association between noninvasive markers of liver fibrosis and QoL.

CONCLUSION

In this cohort, patients with PSC reported high QoL. The PSC PRO score correlated with sCD163 and was sensitive to cholangitis episodes, validating the instrument as a true PSC-specific QoL tool.