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原位肝移植后自身免疫性肝脏疾病及其复发:至今我们学到了什么?
Transpl Int. 2008 Jul 24. [Epub ahead of print]  [2008-8-18]

    肝脏移植后原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)以及自身免疫性肝炎(AIH)都有可能形成复发。rPBC的诊断主要依靠组织学,而rAIH通过血清学、生化以及组织学诊断,而rPSC主要通过组织学和/或胆道成像以及除外其他非吻合因素引起的胆道狭窄后得以诊断。对于复发性疾病(RD)的诊断标准则可能与自体肝脏疾病不同:免疫抑制剂的使用可能改变RD的类型和自然病程,并可能合并有其他导致移植物损伤的原因。RD同样可能存在于正常肝脏检测;报道的发病率主要取决于诊断的方法(尤其是计划性活检)。随着时间的推移RD的危险逐渐增加,但是和移植物的丢失无明显相关性。对于治疗目前没有被证实有特效的方法:熊去氧胆酸能够改善血清学表现并可能减缓rPSC和rPBC的进程;而使用或者增加激素的剂量则可能减缓rAIH的病程。对于rPBC的危险因素包括他克莫司的使用,对于rPSC的危险因素包括无结肠周围移植,而rAIH则可能与一些HLA单倍型相关。

Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) may all recur after liver transplant. Diagnosis of rPBC is defined by histology; rAIH by serology, biochemistry and histology; rPSC by histology and/or imaging of the biliary tree and exclusion of other causes of nonanastomotic biliary strictures. Criteria for recurrent disease (RD) may differ from those used in similar disease in the native liver: frequent use of immunosuppressive therapy changes the pattern and natural history of RD and can co-exist with other transplant-related causes of graft damage. RD may occur in the presence of normal liver tests; the reported incidence will depend on the way in which diagnostic tests (especially protocol biopsies) are applied. The risk of RD increases with time, but does not correlate with the rate of graft loss. Treatment is largely unproven: ursodeoxycholic acid will improve serology and may slow progression of rPSC and rPBC; introduction or increased dose of corticosteroids may reduce progression of rAIH. Risk factors for rPBC include use of tacrolimus compared with cyclosporine; for rPSC include absence of colon peri-transplantation and for rAIH possible associations with some HLA haplotypes have been suggested.


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