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Abstract：
BACKGROUND: Standardization of diagnostic criteria of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) variant syndrome (AIH-PBC VS) has not been achieved so far and evidence-based recommendations for monitoring and treatment of the disease are still lacking. Our study aimed to assess the prevalence, biochemical, and serological features, as well as the clinical course, of VS.
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.
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.
摘要：
背景自身免疫性肝炎（AIH）和原发性胆汁性胆管炎（PBC）变异型综合征（AIH-PBCVS）的诊断标准尚未实现标准化，目前仍缺乏监测和治疗该病的循证建议。我们的研究旨在评估患病率，生物化学，和血清学特征，以及临床过程，VS。
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.
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.
