Adult idiopathic cholestasis: a condition more common in the Canadian Inuit?

• Published in: International Journal of Circumpolar Health
• Main Authors: Gerald Y. Minuk, Galia Pollock, Julia Uhanova
• Format: Article in Journal/Newspaper
• Language: English
• Published: Taylor & Francis Group 2017
• Subjects: Adult idiopathic cholestasis; cholangitis; cholestasis; chronic liver disease; cryptogenic cirrhosis; Inuit; Arctic medicine. Tropical medicine; RC955-962; Arctic; Circumpolar Health; International Journal of Circumpolar Health; inuit
• Online Access: https://doi.org/10.1080/22423982.2017.1388104; https://doaj.org/article/e663e54fadd749669fe7998e928e157a

Despite extensive investigations, some patients have no identifiable cause for their cholestatic liver enzyme abnormalities. The aim of this study was to document the clinical, laboratory, radiologic and histologic features of adult patients with idiopathic cholestasis (AIC). A computerised database of referred patients to a tertiary care hospital outpatient department for assessment of hepatobiliary disorders between 2005 and 2015 was employed to identify and describe features associated with AIC. Of 6,560 patient referrals, sufficient documentation to warrant a diagnosis of AIC was present in 17 (0.26%) cases. Of the 17, a disproportionate number were Canadian Inuit (7/60, 12% Inuit referrals vs. 10/6,500, 0.16% non-Inuit referrals, p<0.0001). The median age of the 17 subjects was 57 years and nine (53%) were female. Clinical and/or laboratory evidence of autoimmune disorders was present in six (35%) cases. Clinical features of hepatic decompensation, radiologic findings in keeping with cirrhosis and histologic confirmation of cirrhosis were present in 47%, 31% and 42% of individuals, respectively. There were no significant improvements in cholestatic liver enzymes and function tests in those treated with ursodiol and/or immunomodulants (n=7) compared to those left untreated (n=10). In conclusion, AIC is a rare condition diagnosed by exclusion. It appears to be more common in the Canadian Inuit population and those with autoimmune disorders. Advanced liver disease is a frequent finding at presentation. Intervention with ursodiol and/or immunomodulants does not appear to be of therapeutic value.