METHODS: We performed a retrospective study including all patients with VS between 1999 and 2020 in four German centers. Data on demographic parameters, biochemical and serological tests, treatment, and outcome were collected.
RESULTS: Of 90 patients (3.1%) meeting Paris criteria for VS diagnosis, 65.6% showed AIH and PBC histological features, while biochemical Paris criteria were observed comparatively rarely. Further antibodies, which were not part of the diagnostic criteria of VS, were found in a subgroup of patients with available data (ACA: 30.0%; anti-CENP-A: 25.0%; anti-CENP-B: 33.3%; anti-SP100: 21.4%). Biochemical response was more frequently observed in patients treated with a combined therapy of ursodeoxycholic acid (UDCA) and immunosuppression (IS). Liver cirrhosis was detected in 31 patients (34.4%) and 25 patients (27.8%) developed clinical manifestations of portal hypertension.
CONCLUSIONS: Biochemical Paris criteria of VS were rarely detected, thus implying that these cut-off values should be redefined. Regarding pharmacological treatment, combined therapy of UDCA and IS appeared to be more effective than monotherapy with UDCA.
方法:我们进行了一项回顾性研究,包括1999年至2020年在四个德国中心的所有VS患者。人口统计参数数据,生化和血清学测试,治疗,并收集了结果。
结果:在符合VS诊断巴黎标准的90例患者(3.1%)中,65.6%显示AIH和PBC组织学特征,而生化巴黎标准观察相对较少。进一步的抗体,这不是VS诊断标准的一部分,在有可用数据的患者亚组中发现(ACA:30.0%;抗CENP-A:25.0%;抗CENP-B:33.3%;抗SP100:21.4%)。在接受熊去氧胆酸(UDCA)和免疫抑制(IS)联合治疗的患者中,更频繁地观察到生化反应。31例(34.4%)患者中发现了肝硬化,25例(27.8%)患者出现了门脉高压的临床表现。
结论:很少检测到VS的生化巴黎标准,因此意味着这些截止值应该重新定义。关于药物治疗,UDCA和IS联合治疗似乎比UDCA单药治疗更有效.