Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone
Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone
复制标题
硬化性胆管炎三种变体的临床特征和自然史的比较：IgG4 相关 SC、PSC/AIH 和单独 PSC。
DOI：
10.1016/j.autrev.2017.05.018
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发表时间：
2017-08-01
影响因子：
13.6
通讯作者：
Ma, Xiong
中科院分区：
文献类型：
--
作者：
Lian, Min;Li, Bo;Ma, Xiong
人们对硬化性胆管炎（SC）的临床变异，即IgG4-SC、PSC/AIH重叠和PSC的兴趣和认识日益增加。在大多数中心，IgG4-SC的特征尚未与其他硬化性胆管炎变体进行彻底的临床比较。此外，PSC/AIH重叠患者相对较少，临床结果不明确，特别是PSC/AIH重叠综合征。我们在此的目的是澄清IgG4-SC、PSC/AIH重叠和PSC单独的自然史的异同。我们还对血清IgG4对IgG4- sc的诊断价值进行了展望，并研究了预测硬化性胆管炎预后的生物标志物。在这项研究中，我们利用我们庞大且定义明确的患者队列进行了一项回顾性队列研究，包括57例IgG4-SC， 36例PSC/AIH重叠患者和55例PSC患者。首先，正如预期的那样，我们注意到免疫球蛋白谱的显著差异，所有患者在就诊时都表现出相似的胆汁淤积谱。57例IgG4-SC患者中有20例发生肝硬化，36例PSC/AIH重叠患者中有15例发生肝硬化，55例PSC患者中有18例发生肝硬化。92.65%的IgG4- sc患者血清IgG4升高，诊断敏感性为86%，特异性为98%。IgG4-SC患者在6个月和1年的治疗反应优于PSC/AIH患者，而后者使用类固醇治疗的反应优于PSC患者。重要的是，与之前的报道不同，IgG4-SC患者的无不良预后生存率降低，因此与PSC/AIH重叠综合征相似。血清IgG和总胆红素分别可用于预测IgG4-SC和PSC/AIH的长期生存。总之，血清IgG4 k 125 ULN对SC患者中IgG4-SC的区分具有良好的可预测性。IgG4-SC似乎是免疫介导的炎症过程，而PSC/AIH重叠更倾向于胆汁淤积性疾病。(C) 2017 Elsevier B.V.版权所有
There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6 month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4 k 125 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease. (C) 2017 Elsevier B.V. All rights reserved.