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肝结节病的见解:组织学模式、肝脏并发症及治疗方法分析

Insights Into Hepatic Sarcoidosis: Analysis of Histological Patterns, Hepatic Complications and Therapeutic Approaches.

作者信息

Horst Ludwig J, Zimmermann Katharina, Lutz Johanna, Weidemann Sören, Lüth Stefan, Lohse Ansgar W, Schulze Zur Wiesch Julian, Schramm Christoph, Wehmeyer Malte H, Müller Martina, Sebode Marcial

机构信息

I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Germany.

出版信息

Liver Int. 2025 Mar;45(3):e70037. doi: 10.1111/liv.70037.

Abstract

BACKGROUND AND AIMS

Sarcoidosis is a granulomatous multi-systemic disorder of uncertain aetiology frequently involving the liver. This study aimed to delineate the histological characteristics, treatment effectiveness and factors predictive of liver-related complications in individuals with hepatic sarcoidosis.

METHODS

This retrospective cohort study included patients diagnosed with hepatic sarcoidosis by liver biopsy, which was conducted at two tertiary care centres from January 2009 to December 2023. We analysed demographic, clinical and laboratory parameters, treatment response and outcome.

RESULTS

We enrolled 70 hepatic sarcoidosis patients with a median follow-up of 45 months (IQR 11-97 months), including 37 males with a median age of 48 years (IQR 37-59 years). Elevated GGT (94%) and ALP (81%) were the most common liver-specific biochemical alterations observed. Using mini-laparoscopy for liver biopsy made it possible to macroscopically identify granulomatous disease in 71% of patients. While at baseline, 16% of the cohort showed evidence of potential portal hypertension, at the last follow-up, 23% of patients developed complications related to portal hypertension. In addition to granulomatous changes, bile duct irregularities were found in 57% of liver biopsies, indicating cholangiopathy being part of the hepatic manifestation of sarcoidosis. Treatment with Ursodeoxycholic acid and prednisolone resulted in a significantly more pronounced decrease in ALT and ALP compared to untreated patients.

CONCLUSIONS

Patients with hepatic sarcoidosis require careful assessment of disease manifestation with a particular focus on portal hypertension. Treatment with UDCA and prednisolone leads to a reduction of biochemical parameters in a significant proportion of these patients.

摘要

背景与目的

结节病是一种病因不明的肉芽肿性多系统疾病,常累及肝脏。本研究旨在描述肝结节病患者的组织学特征、治疗效果及肝脏相关并发症的预测因素。

方法

这项回顾性队列研究纳入了2009年1月至2023年12月在两家三级医疗中心通过肝活检诊断为肝结节病的患者。我们分析了人口统计学、临床和实验室参数、治疗反应及结果。

结果

我们纳入了70例肝结节病患者,中位随访时间为45个月(四分位间距11 - 97个月),其中37例男性,中位年龄48岁(四分位间距37 - 59岁)。观察到最常见的肝脏特异性生化改变为γ-谷氨酰转移酶(GGT)升高(94%)和碱性磷酸酶(ALP)升高(81%)。使用微型腹腔镜进行肝活检使71%的患者能够在宏观上识别肉芽肿性疾病。虽然在基线时,16%的队列显示有潜在门静脉高压的证据,但在最后一次随访时,23%的患者出现了与门静脉高压相关的并发症。除肉芽肿性改变外,57%的肝活检发现胆管不规则,提示胆管病是结节病肝脏表现的一部分。与未治疗的患者相比,熊去氧胆酸和泼尼松龙治疗使谷丙转氨酶(ALT)和ALP的下降更为显著。

结论

肝结节病患者需要仔细评估疾病表现,尤其要关注门静脉高压。熊去氧胆酸(UDCA)和泼尼松龙治疗可使这些患者中的很大一部分生化参数降低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f36/11837985/a612020bfcaa/LIV-45-0-g002.jpg

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