Clinical Impact of Cholestatic Pruritus
The main pathophysiologic manifestation of Alagille syndrome and various forms of progressive familial intrahepatic cholestasis (PFIC) is inadequate bile secretion, with intrahepatic accumulation of bile acids and other potential toxins. What is its clinical impact?
@cally The clinical manifestations may include severe chronic cholestasis manifesting as jaundice, unrelenting pruritus, and subcutaneous lipid deposits secondary to hypercholesterolemia (xanthomas). Intractable pruritus can lead to self-mutilation, scarring, sleep deprivation, and mood disturbance, severely reducing quality of life and often causing family disruption.
Unrelenting pruritus with cholestasis has been reported to have a major impact on the health-related quality of life (HRQOL) of patients and their caregivers. This includes physical and psychosocial domains, as also reported in parent proxy assessments of the HRQOL of their child.
A retrospective, cross-sectional study documented the significant burden on HRQOL among caregivers of those with this condition. Investigators noted impairment in all daily activities, reduced sleep duration, altered work productivity, financial consequences, career challenges, and strained relationships.