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自身免疫性肝炎重叠综合征合并原发性胆汁性胆管炎导致的肝衰竭:一例报告

Liver Failure in Autoimmune Hepatitis Overlap Syndrome With Primary Biliary Cholangitis: A Case Report.

作者信息

Batista Marta, Brito Patricia, Miranda Pedro, Barbosa Sandra, Cotter Jorge

机构信息

Internal Medicine, Hospital Senhora da Oliveira, Guimarães, PRT.

出版信息

Cureus. 2024 Nov 13;16(11):e73574. doi: 10.7759/cureus.73574. eCollection 2024 Nov.

Abstract

Autoimmune hepatitis (AIH) is a complex and long-term liver condition, primarily affecting women, and is marked by high levels of serum gamma globulins, the presence of circulating autoantibodies, and a genetic association. The disease can manifest in a variety of ways, ranging from mild or no symptoms to severe acute liver inflammation. AIH is often associated with other autoimmune disorders, such as primary biliary cholangitis (PBC) and autoimmune thyroiditis. The progression of the disease can lead to cirrhosis or liver failure if not treated with immunosuppressive therapy. Overlap syndromes, such as AIH with PBC, further complicate both diagnosis and management due to the lack of standardized treatment protocols. The rarity of AIH and the absence of large-scale, randomized clinical trials significantly limit current treatment strategies, underscoring the need for ongoing research to improve therapeutic approaches. We report on a 54-year-old female patient who was admitted with acute hepatitis that rapidly progressed to liver failure. After ruling out other causes, corticosteroid treatment was started based on a suspected diagnosis of AIH. Liver biopsy results supported the diagnosis of an overlap syndrome involving AIH and PBC.

摘要

自身免疫性肝炎(AIH)是一种复杂的慢性肝脏疾病,主要影响女性,其特征为血清γ球蛋白水平升高、循环自身抗体的存在以及遗传易感性。该疾病可表现为多种形式,从轻微症状或无症状到严重的急性肝脏炎症。AIH常与其他自身免疫性疾病相关,如原发性胆汁性胆管炎(PBC)和自身免疫性甲状腺炎。如果不进行免疫抑制治疗,疾病进展可导致肝硬化或肝衰竭。重叠综合征,如AIH合并PBC,由于缺乏标准化治疗方案,会使诊断和管理更加复杂。AIH的罕见性以及缺乏大规模随机临床试验显著限制了当前的治疗策略,凸显了持续开展研究以改进治疗方法的必要性。我们报告了一名54岁女性患者,她因急性肝炎入院,病情迅速进展为肝衰竭。排除其他病因后,基于疑似AIH的诊断开始使用皮质类固醇治疗。肝活检结果支持了AIH与PBC重叠综合征的诊断。

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本文引用的文献

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