Liver biopsy in primary biliary cholangitis: is sinusoidal fibrosis the missing key?
Liver biopsy in primary biliary cholangitis: is sinusoidal fibrosis the missing key?
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DOI：
10.1136/jclinpath-2019-205958
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发表时间：
2019-10-01
影响因子：
3.4
通讯作者：
McMahon, Raymond F.
中科院分区：
文献类型：
--
作者：
Warnes, Thomas;Roberts, Stephen;McMahon, Raymond F.
目的肝活检在原发性胆道性胆管炎（PBC）中的作用是有争议的，也是组织学评估的最佳方法。我们比较了Ludwig和Ishak分期系统以及日本（Nakanuma）分期系统的三个组成部分，以评估其临床和生化相关性以及预后价值。方法我们回顾了106例PBC患者的活检，这些患者来自于先前的秋水仙碱治疗试验，随访24-34年，随后评估了5项临床结果：肝代偿失调、胆汁淤积性PBC死亡/肝移植、门脉高压PBC死亡、所有PBC死亡和总生存。结果Ludwig和Ishak分期与血清胆红素、Mayo评分和Child评分等预后参数相关。血清天冬氨酸转氨酶与界面肝炎（IFH）有关，碱性磷酸酶与胆蛋白沉积、胆管损失和胆汁淤积有关。Ludwig与所有五个临床结果相关，而Ishak分期仅与两个显著相关。虽然肝窦纤维化、orcein沉积、BD损失和胆汁淤积均可预测肝死亡/移植，但经Mayo评分校正后，预测临床结果的唯一组织学参数是IFH（与两项相关）和窦状纤维化（与所有五项相关）。结论约20%的PBC患者需要肝活检。Ludwig系统比Ishak和Nakanuma分期系统的三个单独组成部分中的任何一个都具有更高的预后价值，但除了Mayo评分之外，提供独立预后价值的主要组织学参数是窦状纤维化。
Aims The role of liver biopsy in primary biliary cholangitis (PBC) is controversial, as is the optimal method of histological assessment. We compared the Ludwig and Ishak systems and three components of the Japanese (Nakanuma) staging system to evaluate their clinical and biochemical correlations and prognostic value. Methods We reviewed biopsies from 106 patients with PBC, derived from a previous trial of colchicine therapy with 24-34 years' follow-up, following which five clinical outcomes were evaluated: hepatic decompensation, cholestatic PBC death/liver transplant, portal hypertensive PBC death, all PBC deaths and overall survival. Results Ludwig and Ishak stages correlated well with prognostically significant parameters, including serum bilirubin, and both Mayo and Child Scores. Serum aspartate aminotransferase correlated with interface hepatitis (IFH), and alkaline phosphatase with orcein deposition, bile duct (BD) loss and cholestasis. Ludwig correlated with all five clinical outcomes, while Ishak stage was only significantly correlated with two. While sinusoidal fibrosis, orcein deposition, BD loss and cholestasis all predicted hepatic death/transplant, after correction for Mayo Score, the only histological parameters predictive of clinical outcomes were IFH (associated with two) and sinusoidal fibrosis (associated with all five). Conclusion Liver biopsy is required in the diagnosis of around 20% of patients with PBC. The Ludwig system is of more prognostic value than both Ishak and any of the three individual components of the Nakanuma staging system, but the major histological parameter providing independent prognostic value beyond the Mayo Score is sinusoidal fibrosis.