Tanaka Atsushi, Mertens Joachim C
Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
University Hospital Zurich, Zurich, Switzerland.
Inflamm Intest Dis. 2016 Apr;1(1):9-14. doi: 10.1159/000445259. Epub 2016 Apr 1.
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.
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.
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.
原发性硬化性胆管炎（PSC）是一种病因不明的慢性胆汁淤积性肝病，是炎症性肠病（IBD）尤其是溃疡性结肠炎（UC）的重要肝胆并发症。进一步细分时，约3 - 8%的UC患者患有PSC，而在克罗恩病患者中，报道的PSC患病率可能在1%至3.5%之间。尽管日本报道UC中PSC的发生率仅为34%，但同一登记数据表明，即使在日本，年轻PSC患者中的患病率也高达57%，这与欧美60 - 80%的患病率相当。此外，PSC患者的UC临床特征与无PSC患者不同，例如直肠不受累和右侧优势。
非典型IBD与PSC之间的紧密联系表明，PSC的发病机制涉及肠道病理，包括异常的肠道微生物群和黏膜淋巴细胞的异常激活。与典型UC的病理相比，UC合并PSC的情况似乎有所不同。
解决“东西方之间是否存在差异？”这一问题的关键在于基因研究，尤其是PSC的全基因组关联研究，该研究已在西方开展，在东方也非常有必要进行。
