Abstract：
Liver dysfunction in systemic lupus erythematosus (SLE) is caused by disease activity or secondary conditions like coexistent autoimmune liver diseases. In Taiwan, despite sporadically reported cases of SLE-autoimmune hepatitis (AIH) overlap disease, larger-scale monocentric investigations for such overlapping patients are not available. Retrospective analyses were performed in a hospitalized SLE cohort with 805 patients for identifying co-existent AIH from 2014 to 2023, focusing on distinct therapeutic modalities and differential diagnosis between SLE-AIH overlap and lupus hepatitis (LH). There were 5 cases (a 0.6% occurrence), all females aged 25-58 years (44 ± 13). Ages for the SLE diagnosis were 19-51 years (30 ± 13), while ages for the AIH diagnosis were 22-57 years (36 ± 14). Contradictory to interface hepatitis in SLE-AIH overlap, liver biopsy only demonstrated non-specific abnormalities in LH. Liver cirrhosis was identified in SLE-AIH overlap but not in LH. After corticosteroids/azathioprine therapy, there were normalized liver function in all LH. In 2 SLE-AIH overlap cases refractory to such therapy, one received B-cell depletion therapy (annual rituximab infusion, 375 mg/m2 weekly × 4) and another accepted living-donor liver transplantation from sibling due to advanced liver cirrhosis, leading to improved hepatic dysfunction in both.
摘要：
系统性红斑狼疮（SLE）的肝功能障碍是由疾病活动或继发性疾病引起的，例如共存的自身免疫性肝病。在台湾,尽管偶尔报道的SLE-自身免疫性肝炎（AIH）重叠疾病的病例，对于此类重叠患者,无法进行更大规模的单中心检查.在2014年至2023年的805例住院SLE队列中进行了回顾性分析，以确定共存的AIH，重点是SLE-AIH重叠和狼疮肝炎（LH）之间的不同治疗方式和鉴别诊断。有5例（0.6％的发生率），所有女性年龄25-58岁（44±13）。诊断为SLE的年龄为19-51岁（30±13），而AIH诊断的年龄为22-57岁（36±14）。与SLE-AIH重叠的界面肝炎矛盾，肝活检仅显示LH的非特异性异常。在SLE-AIH重叠中发现肝硬化，但在LH中未发现。皮质类固醇/硫唑嘌呤治疗后，所有LH患者肝功能均正常。在2例此类治疗难以治疗的SLE-AIH重叠病例中，1人接受B细胞耗竭治疗（每年输注利妥昔单抗，375mg/m2每周×4）和另一个接受的活体供体肝移植由于晚期肝硬化的同胞，导致改善肝功能障碍。
