Prospective Evaluation of PBC-specific Health-related Quality of Life Questionnaires in Patients with Primary Sclerosing Cholangitis

Background & Aims: Primary biliary cirrhosis and Primary sclerosing cholangitis are autoimmune cholestatic liver diseases sharing a lot in common, including a significant impairment of patients' health-related quality of life HRQoL HRQoL in PBC is assessed with disease-specific PBC-40 and PBC-27 questionnaires. A PSC-specific questionnaire has not been developed. Neither PBC-40 nor PBC-27s applicability for PSC has been evaluated. We applied these three questionnaires for HRQoL assessment in a large homogenous cohort of PSC patients.

Patients And Methods: This cross-sectional study enrolled 102 Caucasian PSCs and 53 matched healthy controls and measured HRQoL using generic SF-36, and disease-specific (PBC-40/PBC-27) questionnaires.

Results: (i) SF-36. Most SF-36 domains were significantly lower in PSCs than controls. Physical Functioning and Mental Component Summary scores were significantly lower in female patients and correlated negatively with age but not with concurrent inflammatory bowel disease. Cirrhosis was associated with lower Physical Functioning, Role Physical, General Health, Vitality and Physical Component Summary. (ii) PBC-40 and PBC-27. Both tools showed similar HRQoL impairment scoring. Fatigue and Cognitive were impaired in female patients. Several correlations existed between HRQoL and laboratory parameters, including cholestatic tests and Itch. Cirrhosis correlated with Other symptoms and Fatigue PBC-40. (iii) PBC-40 vs PBC-27. Strong correlations among most domains of both questionnaires were seen, as well as between (iv) SF-36 vs PBC-40 or SF-36 vs PBC-27.

Conclusion: This is the first study directly comparing PBC-40, PBC-27 and SF-36 in PSC. PSC patients, especially females, show HRQoL impairment. PBC-40 and PBC-27 questionnaires could be of potential use for HRQoL assessment in PSC.