Division of Rheumatology and Immunology, Department of Internal Medicine, Taiwan.
Department of Pathology, Taiwan.
J Formos Med Assoc. 2024 Oct;123(10):1110-1114. doi: 10.1016/j.jfma.2024.06.003. Epub 2024 Jun 8.
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/m weekly × 4) and another accepted living-donor liver transplantation from sibling due to advanced liver cirrhosis, leading to improved hepatic dysfunction in both.
系统性红斑狼疮(SLE)患者的肝功能障碍可由疾病活动或共存的自身免疫性肝病等继发情况引起。在台湾,尽管有散发性报道的SLE-自身免疫性肝炎(AIH)重叠疾病病例,但尚无此类重叠患者的更大规模单中心调查。对 2014 年至 2023 年间住院 SLE 队列中的 805 例患者进行了回顾性分析,以确定是否存在共存的 AIH,重点关注不同的治疗方式和 SLE-AIH 重叠与狼疮性肝炎(LH)的鉴别诊断。共发现 5 例(发生率为 0.6%),均为 25-58 岁的女性(44±13 岁)。SLE 的诊断年龄为 19-51 岁(30±13 岁),AIH 的诊断年龄为 22-57 岁(36±14 岁)。与 SLE-AIH 重叠中的界面性肝炎相反,LH 仅表现为非特异性肝组织学异常。在 SLE-AIH 重叠中发现了肝硬化,但在 LH 中没有。在接受皮质类固醇/硫唑嘌呤治疗后,所有 LH 患者的肝功能均恢复正常。在 2 例对这种治疗有抗药性的 SLE-AIH 重叠患者中,1 例接受了 B 细胞耗竭治疗(每年利妥昔单抗输注,375mg/m 每周×4),另 1 例由于肝硬化进展接受了来自同胞的活体供肝移植,这 2 例患者的肝功能障碍均得到改善。
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