Tanaka Atsushi, Tazuma Susumu, Okazaki Kazuichi, Tsubouchi Hirohito, Inui Kazuo, Takikawa Hajime
Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
J Hepatobiliary Pancreat Sci. 2014 Jan;21(1):43-50. doi: 10.1002/jhbp.50. Epub 2013 Oct 29.
We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement.
The design was a questionnaire-based, multi-center retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005.
We enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4-SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC.
We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
我们之前在日本开展了原发性硬化性胆管炎（PSC）的全国性调查，并证实了日本PSC患者的几个特征，但IgG4相关性硬化性胆管炎（IgG4-SC）患者可能会被误诊为PSC。自2012年IgG4-SC的临床诊断标准确立以来，我们再次进行了全国性调查，以研究无胰腺受累的PSC和IgG4-SC的特征。
本研究为基于问卷调查的多中心回顾性研究。纳入的研究对象为2005年后诊断的无胰腺受累的PSC和IgG4-SC患者。
我们纳入了197例无胰腺病变的PSC患者和43例IgG4-SC患者。IgG4-SC患者中男性占主导地位显著（P = 0.006）。在PSC患者中，明显观察到年龄分布的两个高峰。在任何年龄小于45岁的患者中均未检测到IgG4-SC。就诊时，PSC患者的血清白蛋白和IgM显著更高，而IgG4-SC患者的血清IgG和IgG4显著升高。仅在68/197例PSC患者（34%）中检测到炎症性肠病（IBD）。IgG4-SC的预后明显好于PSC。
我们证实了日本PSC患者的几个有趣的临床细节：年龄分布的两个高峰以及IBD的较低患病率。
