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Title: [Clinical observation of isolated immunoglobulin G4-related sclerosing cholangitis and immunoglobulin G4 sclerosing cholangitis combined autoimmune pancreatitis].
Author: Jiang P, Lian M, Li B, Xiao X, Miao Q, Wang QX, Ma X.
Journal: Zhonghua Gan Zang Bing Za Zhi; 2018 Jun 20; 26(6):415-419. PubMed ID: 30317753.
Abstract:
Objective: To compare and analyze patient's general condition, laboratory testing and therapeutic responses of isolated immunoglobulin G4- related sclerosing cholangitis (IgG4-SC) and immunoglobulin G4 sclerosing cholangitis combined autoimmune pancreatitis (IgG4-SC/AIP). Methods: A retrospective study was conducted on IgG4-SC patients who attended outpatient and inpatients department of our hospital from April 2014 to March 2018 and their demographic characteristics, laboratory testing, and therapeutic responses were statistically analyzed. Normal distribution of continuous variables was compared with t-test, non-normal distribution of continuous variables was compared using the Mann-Whitney U test, and the categorical variables were compared with χ (2) test. Results: 29 IgG4-SC patients were included, including 19-isolated IgG4-SC and 10 IgG4-SC combined AIP (IgG4-SC/AIP). The average age of onset in the isolated IgG4-SC group was (46.06±19.03) years which was lower than IgG4-SC/AIP group (62.60±15.11), t = -2.360, P < 0.05. The median IgG4 in IgG4-SC/AIP patients is higher than that in isolated IgG4-SC, respectively 10.87 (3.73 ~ 20.13) and 3.14 (2.37 ~ 4.78)g/L(U = 159.000, P < 0.05). IgG4/IgG ratio is higher in IgG4-SC/AIP, than that in isolated IgG4-SC, respectively 0.62(0.23 ~ 0.86) and 0.16(0.10 ~ 0.21), U = 130.000, P < 0.05. Liver cirrhosis was more common in isolated IgG4-SC group (47%) than the IgG4-SC/AIP group (0), χ (2) = 9.637, P < 0.05. The median biochemical response time of isolated IgG4-SC group was 3.00 (2.00 to 4.00) months, which was longer than 1.00 (1.00 to 1.25) months of IgG4-SC/AIP group, U = 30.000, P < 0.05. The biochemical recurrence rate of isolated IgG4-SC group was 32%, which was lower than that of IgG4-SC/AIP (χ (2) = 6.461, P < 0.05). Conclusion: Serum IgG4 level and IgG4/IgG ratio were higher in patients with IgG4-SC/AIP group, and therapeutic responses in isolated IgG4-SC patients were worse than that of IgG4-SC/AIP patients. The efficacy of glucocorticoid monotherapy and immunosuppressive agents combined with glucocorticoid therapy demonstrated no considerable difference in IgG4-SC patients. 目的： 比较分析单独免疫球蛋白G4相关硬化性胆管炎（IgG4-SC）与IgG4-SC合并自身免疫性胰腺炎（AIP）患者的一般情况、实验室检查指标和治疗应答情况。 方法： 采用回顾性研究，以2014年4月至2018年3月至本院门诊就诊及住院的IgG4-SC患者为研究对象，统计分析其人口学特点、实验室检查指标和治疗应答情况。正态分布的连续变量资料比较采用t检验，非正态分布的连续变量资料比较采用Mann-Whitney U检验，分类变量资料的比较采用χ(2)检验。 结果： 共纳入IgG4-SC患者29例，其中单独IgG4-SC 19例、IgG4-SC合并AIP （IgG4-SC/AIP）10例。单独IgG4-SC组平均起病年龄为（46.06±19.03）岁，低于IgG4-SC/AIP组的（62.60±15.11）岁，t = -2.360，P<0.05。IgG4-SC/AIP组患者中位血清IgG4水平为10.87（3.73~20.13）g/L，高于单独IgG4-SC组的3.14（2.37~4.78）g/L，U = 159.000，P<0.05。IgG4-SC/AIP组中位IgG4/IgG比值高于单独IgG4-SC组，分别为0.62（0.23~0.86）、0.16（0.10~0.21），U = 130.000，P<0.05。单独IgG4-SC组患者肝硬化患者（47%）多于IgG4-SC/AIP组（0），χ(2) = 9.637，P<0.05。单独IgG4-SC组患者中位生物化学应答时间为3.00（2.00~4.00）个月，长于IgG4-SC/AIP组的1.00（1.00~1.25）个月，U = 30.000，P<0.05。单独IgG4-SC组患者生物化学指标复常率为32%，低于IgG4-SC/AIP组的80%（χ(2) = 6.461，P<0.05）。 结论： IgG4-SC合并AIP组患者的血清IgG4水平、IgG4/IgG比值更高，单独IgG4-SC患者的治疗应答可能较IgG4-SC/AIP患者的差，免疫抑制剂联合糖皮质激素治疗与糖皮质激素单药治疗的效果在IgG4-SC患者中未见明显差异。.

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