Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin and an important hepatobiliary complication of inflammatory bowel diseases (IBD), especially ulcerative colitis (UC). When further differentiated, about 3-8% of UC patients suffer from PSC, whereas among Crohn's disease patients the reported prevalence of PSC is probably between 1 and 3.5%. Although it was reported from Japan that the frequency of PSC in UC was only 34%, the same registry data indicated that the prevalence was up to 57% among young patients with PSC even in Japan, which is comparable to the 60-80% in Europe and the US. Additionally, the clinical features of UC in patients with PSC are different from those in patients without PSC, for instance, rectal sparing and right-sided dominance.
Summary: The strong link between atypical IBD and PSC suggests that the pathogenesis of PSC involves pathology of the gut, including abnormal gut microbiota and aberrant activation of mucosal lymphocytes. These seem to be different in UC PSC as compared to the pathology of typical UC.
Key messages: The key to solving the question 'Is there a difference between East and West?' are genetic studies, genome-wide association studies of PSC in particular, which have already been performed in the West and are strongly warranted in the East.
Keywords: Primary sclerosing cholangitis; Ulcerative colitis.
Contrasting Pattern of Chronic Inflammatory Bowel Disease in Primary and Autoimmune Sclerosing CholangitisI Bjarnason et al. EBioMedicine 2 (10), 1523-7. PMID 26629548.Collectively these findings lend support to the suggestion that the chronic inflammatory bowel disease associated with PSC and in particular AISC may represent a distinct …
Primary Sclerosing Cholangitis Is Associated With a Distinct Phenotype of Inflammatory Bowel DiseaseK Boonstra et al. Inflamm Bowel Dis 18 (12), 2270-6. PMID 22407885.IBD in PSC patients represents a distinct phenotype in that pancolitis is observed in 94% of PSC-UC and colitis in 96% of PSC-CD patients. Backwash ileitis and rectal spa …
Th1 and Innate Lymphoid Cells Accumulate in Primary Sclerosing Cholangitis-associated Inflammatory Bowel DiseaseA Gwela et al. J Crohns Colitis 11 (9), 1124-1134. PMID 28383652.Our study suggests that PSC-UC represents a different immunological disorder from UC, characterised by increased intestinal Th1 and ILC responses. These results provide f …
Primary Sclerosing Cholangitis: A Clinical UpdateKD Williamson et al. Br Med Bull 114 (1), 53-64. PMID 25981516. - ReviewOn the basis of the genetic studies discussed earlier, novel agents are being developed and trialled in the treatment of PSC.
Distinctive Inflammatory Bowel Disease Phenotype in Primary Sclerosing CholangitisAB de Vries et al. World J Gastroenterol 21 (6), 1956-71. PMID 25684965. - ReviewIBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn's disease and therefore requires specialized management.