Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study

Background & Aims Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap.Methods We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.Results N = 1275 patients were included (PBC: n = 342, PBC-AIH: n = 160, PSC: n = 305, PSC-AIH: n = 121, AIH: n = 347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: Delta R2 = 0.012, p = 0.041; mental: Delta R2 = 0.016, p = 0.020), but not with PBC (physical: Delta R2 = 0.008, p = 0.081; mental: Delta R2 = 0.001, p = 0.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity.Conclusions Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.