Introduction Autoimmune sclerosing cholangitis is the paediatric term applied to children presenting with features of autoimmune hepatitis and sclerosing cholangitis. We hypothesised that if this inflammatory phenotype was a continuum, young adults with primary sclerosing cholangitis (PSC) would also have a more inflammatory presentation.
Methods We undertook a retrospective case-note review of our patients with an established diagnosis of PSC presenting between 2003–2013 (n = 116). Clinical characteristics and laboratory parameters were collected, and differences in disease phenotype correlated with age at presentation (SPSSv21).
Results The median age of disease presentation in our cohort was 44 years (IQR:25–56). Although there was no significant correlation between patient age and mode of disease presentation, younger age was more commonly associated with lower baseline serum ALP (Spearman’s rho = 0.239; P = 0.011). Patient age negatively correlated with ALP:AST ratio (rho = 0.252; P = 0.008); however, there was no correlation with serum AST, bilirubin, albumin, platelet count, INR, IgG titre or ANA/ASMA status. Using quartile cut-points in order to compare extremes of age, individuals presenting below the age of 25 (Q4; 7.6; 3.2–13.0) (P = 0.023). Age <25 at disease presentation was more often associated with an ALP:AST ratio <1.5 (11/25 [44%] vs. 4/25; [16%], P = 0.017). There were no significant differences in IBD phenotype, number of patients meeting transplantation or median time to transplant.
Conclusion Younger patients more commonly have a lower ALP/AST ratio at disease presentation, and may indicate a more ‘inflammatory’ PSC phenotype.
Disclosure of Interest None Declared.
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