目的:结节病肝脏受累的临床表现可以从无症状疾病到严重并发症如肝硬化和门脉高压。然而,关于肝结节病的数据有限,缺乏基于证据的建议。我们的研究旨在评估以高加索人群为主的肝结节病的特征和临床过程。
方法:我们进行了一项回顾性研究,包括2004年至2020年在5个德国中心的所有肝结节病患者。中位随访时间为36个月(0.0-195)。人口统计参数数据,临床表现,诊断测试结果,治疗,并收集了结果。
结果:共发现1,476例结节病患者和62例肝脏受累患者(4.2%)。在患者中,51.6%是女性,80.6%是白种人。大多数患者无症状,并且被观察到具有肝酶升高的胆汁淤积模式。9例患者(14.5%)出现肝硬化,其中6人出现门静脉高压症的临床表现。54名患者接受了药物治疗,最常见的是糖皮质激素(69.4%)或熊去氧胆酸(UDCA)(40.3%)。在接受糖皮质激素治疗的患者中,碱性磷酸酶(ALP)水平从基线平均降低了60.8%,在接受UDCA治疗的患者中,碱性磷酸酶(ALP)水平降低了59.9%。17名患者接受了二线药物的治疗增强,其中8例患者在随访期间ALP水平恢复正常。所有患者均未接受肝移植或发展为肝细胞癌(HCC)。其中三名患者在随访期间因肝脏相关并发症死亡。
结论:结节病患者中发现结节病肝受累4.2%,其中14.5%具有临床意义。这些发现突出了早期识别的重要性,监测,治疗肝结节病,鉴于其与终末期肝病相关的死亡率增加。
背景:结节病肝受累的临床诊断和监测尚未标准化,肝脏受累的管理是一个临床挑战,因为它在许多方面的特征仍然很差。我们的结果表明,三分之一的肝结节病患者表现为临床上显著的门脉高压,14.5%患有肝硬化,3例患者因肝脏相关并发症死亡。关于药物治疗选择,皮质类固醇和UDCA是最常用的药物,两者都被证明能在大多数患者中诱导生化反应。这些发现突出了正确和早期识别结节病肝脏受累的重要性。因为潜在的进行性疾病过程。
OBJECTIVE: Clinical manifestation of hepatic involvement in sarcoidosis can vary from asymptomatic disease to severe complications such as cirrhosis and portal hypertension. However, data on hepatic sarcoidosis are limited, and evidence-based recommendations are lacking. Our study aimed to assess the features and clinical course of hepatic sarcoidosis in a predominantly Caucasian cohort.
METHODS: We performed a retrospective study including all patients with hepatic sarcoidosis between 2004 and 2020 in 5 German centres. The median follow-up time was 36 months (range 0.0-195). Data on demographic parameters, clinical manifestations, diagnostic test results, treatment, and outcome were collected.
RESULTS: A total of 1,476 patients with sarcoidosis and 62 patients with hepatic involvement (4.2%) were identified. Of the patients, 51.6% were female, and 80.6% were Caucasian. Most patients were asymptomatic and were observed to have a cholestatic pattern of liver enzyme elevations. Cirrhosis was detected in 9 patients (14.5%), of whom 6 developed clinical manifestations of portal hypertension. Fifty-four patients were medically treated, most commonly with glucocorticoids (69.4%) or ursodeoxycholic acid (UDCA) (40.3%). Levels of alkaline phosphatase (ALP) decreased by 60.8% on average from baseline in patients treated with glucocorticoids and by 59.9% in patients treated with UDCA. Seventeen patients received treatment augmentation with a second line agent, of whom 8 patients normalised ALP levels during follow-up. None of the patients underwent liver transplantation or developed hepatocellular carcinoma (HCC). Three of the patients died during follow-up owing to liver-related complications.
CONCLUSIONS: Hepatic involvement in sarcoidosis was found in 4.2% of patients with sarcoidosis and was clinically significant in 14.5% of those. These findings highlight the importance of early identifying, monitoring, and treating hepatic sarcoidosis, given its increased mortality when associated with end-stage liver disease.
BACKGROUND: Clinical diagnostic and surveillance of hepatic involvement in sarcoidosis has not been standardised, and management of hepatic involvement is a clinical challenge, since it remains poorly characterised in many ways. Our results show that one-third of patients with hepatic sarcoidosis presented with clinically significant portal hypertension, 14.5% suffered from cirrhosis, and 3 patients died owing to liver-related complications. Regarding pharmacological treatment options, corticosteroids and UDCA were the medical agents most frequently used, and both of them have been shown to induce biochemical response in the majority of patients. These findings highlight the importance of correctly and early identifying hepatic involvement in sarcoidosis, because of the potentially progressive course of disease.