原发性硬化性与IgG4相关硬化性胆管炎临床特征比较

Comparing the clinical characteristics of IgG4-related sclerosing cholangitis with primary sclerosing cholangitis

目的比较IgG4相关性硬化性胆管炎（IgG4-SC）和原发性硬化性胆管炎（PSC）的临床特征。方法回顾分析2004年1月至2014年12月北京协和医院住院治疗的72例IgG4．SC患者以及32例PSC患者的临床特征。结果32例PSC患者中男女各16例。72例IgG4-SC患者中男性61例，女性11例，男女比例为5．5：1。就诊时两组患者的平均年龄分别为44．9岁（11～77岁）和59．8岁（28—83岁，P〈0．05）。PSC和IgG4-SC最常见的临床症状均为腹痛（50．0％比68．1％）和黄疸（78．1％比81．9％）。IgG4-SC患者血清IgG4水平显著高于PSC患者（P〈0．05）。IgG4-SC患者血清总蛋白水平高于PSC患者，差异具有统计学意义（P〈0．05）。IgG4．SC患者超声内镜发现52例（52／57，91．2％）胆管增厚，其检出率明显高于腹部超声（7／61，11．5％）和腹部CT（24／72，33．3％）（P〈0．05）。PSC患者随访超过2年的有12例，其中2例保守治疗无效选择肝移植，5例死于肝衰竭及感染，3例病情稳定。随访超过2年的IgG4-SC患者共43例，其中16例复发，复发率为37．2％（16／43），胆管外受累器官较多或胆管受累节段较多患者复发率较高。结论PSC和IgG4-SC皆属胆汁淤积性肝病。二者临床和影像学表现虽有诸多相似之处，但仍有着各自独特的临床特点。因IgG4．sc患者对激素治疗敏感，故预后良好。因此，PSC与IgG4-SC两者的鉴别十分重要。

Objective To analyze and compare the clinical characteristics of primary sclerosing cholangitis （PSC） with IgG4-related sclerosing cholangitis （IgG4-SC）. Methods The clinical data of 32 PSC patients and 72 IgG4-SC patients who were hospitalized in Peking Union Medical College Hospital （PUMCH） from January 2004 to December 2014 were retrospectively analyzed. Results Of the 32 PSC patients, there were 16 male and 16 female. Of the 72 IgG4-SC patients, there were 61 male and 11 female, （ ratio = 5.5：1 ）. The average ages were 44.9 （ 11 - 77 ） and 59.8 （ 28 - 83 ） years, respectively （ P 〈 0. 05）. The most common symptoms of PSC and IgG4-SC were abdominal pain and jaundice, and the incidences of abdominal pain and jaundice were 50.0% and 68.1%, 78.1% and 81.9%, respectively. The serum IgG4 level of the IgG4-SC patients was significantly higher than the PSC patients （P 〈 0.05 ）. The total protein in serum of the IgG4-SC patients was higher than the PSC patients （P 〈 0. 05）. The rate of bile duct wall thickening as detected on endoscopic uhrasonography （EUS） was higher than by abdominal ultrasound and abdominal CT, which were 91.2%, 11.5% and 33.3%, respectively （P 〈 0.05 ）. 12 PSC patients were followed up for over 2 years, including 2 patients who underwent liver transplantation after failure of conservative treatment, 5 patients who died from hepatic failure and infection, and 3 with stable condition, 43 IgG4-SC patients were followed up for over 2 years, including 16 patients with relapse. The recurrence rate was 37.2% （16/43）. The more the extrabiliary organs or bile duct segments were involved, the higher was the recurrence rate. Conclusions Both PSC and IgG4-SC are cholestatic diseases, and they have many similarities in clinical and imaging manifestations. However, they still have unique features. IgG4-SC is sensitive to glucocorticoids therapy and has good prognosis. Thus, it is important to differentiate PSC from IgG4-SC.