Longitudinal, Multicenter Study of Clinical Factors Impacting Health-Related Quality of Life in Pediatric Autoimmune Liver Disease

Abstract

Background: Children with autoimmune liver disease (AILD) face unique challenges that may impair their health-related quality of life (HRQoL). This multicenter, prospective, longitudinal study evaluated HRQoL over time and identified associated clinical factors.

Methods: A total of 162 participants from five centers completed at least one HRQoL assessment. Medical and laboratory data were abstracted within three months of each assessment. Fatigue and pruritus were reported at one center. Generalized linear mixed-effects modeling was used to examine longitudinal associations between HRQoL and clinical variables.

Results: Participants reported the lowest HRQoL scores in school and emotional domains, while the physical and social domains were less affected. Compared to healthy children, participants with AILD reported lower overall HRQoL. Longitudinal analysis revealed that caregivers of participants with overlap syndrome reported higher emotional, social, psychosocial, and total scores. Paradoxically, children with disease complications had better school scores, possibly due to increased support services. Prednisone use was associated with improved emotional scores, while azathioprine use was associated with lower social scores. Elevated ALT levels were associated with lower HRQoL scores, particularly when reported by caregivers. Disease duration and presence of inflammatory bowel disease were not significantly associated with HRQoL.From one center, fatigue and pruritus were significantly associated with lower HRQoL, especially in the physical, psychosocial, and total domains. Fatigue was also associated with elevated liver enzymes and reduced rates of biochemical remission, suggesting that inflammation may contribute to a disrupted liver-brain axis.

Conclusion: These findings underscore the multidimensional impact of AILD on pediatric patients and highlight the need for further research into the pathophysiology of fatigue and potential therapeutic interventions.