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移植后复发性原发性胆汁性肝硬化、原发性硬化性胆管炎和自身免疫性肝炎。

Recurrent primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis after transplantation.

作者信息

Faust T W

机构信息

Liver Study Unit, Section of Gastroenterology, Department of Medicine, University of Chicago Hospitals and Clinics, University of Chicago, 5841 South Maryland Avenue, MC4076, Chicago, IL 60637, USA.

出版信息

Semin Liver Dis. 2000;20(4):481-95. doi: 10.1055/s-2000-13157.

DOI:10.1055/s-2000-13157
PMID:11200417
Abstract

Viral hepatitis and malignancy frequently recur after transplantation, but recurrence of primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis is controversial. Differences in study design, number of patients, immunosuppressive treatment, length of follow-up, and criteria for recurrence account for discrepant results. Most patients with suspected recurrent disease are asymptomatic after transplantation. In patients transplanted for PBC, antimitochondrial antibodies frequently persist and do not correlate with disease recurrence; liver biopsy remains the gold standard for diagnosis. Exclusion of other disorders that can mimic PBC is paramount prior to making a diagnosis of recurrent disease. The effects of immunosuppression may modify or delay disease expression within the graft. If PBC recurs, intermediate-term patient and graft survival is excellent, but long-term studies will be necessary to address the impact of disease recurrence on the allograft. Due to lack of a diagnostic gold standard, a diagnosis of recurrent PSC after transplantation is difficult to make. An accurate diagnosis of PSC recurrence requires well-defined cholangiographic and histologic criteria. Other disorders that can produce biliary strictures after transplantation should be excluded. As with PBC, the effects of immunosuppression may modify or delay disease expression within the graft; medium-term patient and graft survival is excellent. Recurrence of autoimmune hepatitis is based on clinical, biochemical, serologic, and histologic criteria. As in patients transplanted for PBC and PSC, other conditions that can mimic autoimmune hepatitis require exclusion prior to making a diagnosis of recurrence. Most adult recipients respond to an increase in immunosuppression, whereas pediatric recipients do not respond as well. A cautious approach to withdrawal of immunosuppression is warranted in all patients transplanted for autoimmune hepatitis and the consequences of recurrent disease within the graft will require prolonged follow-up. Future studies should focus on preventive and therapeutic strategies for recurrent autoimmune diseases after transplantation.

摘要

病毒性肝炎和恶性肿瘤在移植后常复发,但原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)和自身免疫性肝炎的复发存在争议。研究设计、患者数量、免疫抑制治疗、随访时间以及复发标准的差异导致了结果的不一致。大多数疑似疾病复发的患者在移植后无症状。对于因PBC接受移植的患者,抗线粒体抗体常持续存在,且与疾病复发无关;肝活检仍是诊断的金标准。在诊断复发性疾病之前,排除其他可模拟PBC的疾病至关重要。免疫抑制的影响可能会改变或延迟移植物内疾病的表现。如果PBC复发,中期患者和移植物存活率良好,但需要长期研究来探讨疾病复发对同种异体移植物的影响。由于缺乏诊断金标准,移植后复发性PSC的诊断很难做出。PSC复发的准确诊断需要明确的胆管造影和组织学标准。应排除移植后可导致胆管狭窄的其他疾病。与PBC一样,免疫抑制的影响可能会改变或延迟移植物内疾病的表现;中期患者和移植物存活率良好。自身免疫性肝炎的复发基于临床、生化、血清学和组织学标准。与因PBC和PSC接受移植的患者一样,在诊断复发之前需要排除其他可模拟自身免疫性肝炎的疾病。大多数成年受者对免疫抑制增加有反应,而儿科受者反应较差。对于所有因自身免疫性肝炎接受移植的患者,谨慎减少免疫抑制是必要的,移植物内复发性疾病的后果需要长期随访。未来的研究应聚焦于移植后复发性自身免疫性疾病的预防和治疗策略。

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Hepatology. 2021 Oct;74(4):2047-2057. doi: 10.1002/hep.31911. Epub 2021 Sep 9.
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Clin Rev Allergy Immunol. 2015 Jun;48(2-3):273-84. doi: 10.1007/s12016-014-8457-4.
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Diagnosis, pathogenesis, and treatment of autoimmune hepatitis after liver transplantation.
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Dig Dis Sci. 2012 Sep;57(9):2248-66. doi: 10.1007/s10620-012-2179-3. Epub 2012 May 6.