Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Pathology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Semin Liver Dis. 2018 Aug;38(3):284-297. doi: 10.1055/s-0038-1666853. Epub 2018 Jul 24.
Sarcoidosis is a multisystem inflammatory disease, which is characterized by the development of noncaseating granulomas amid healthy tissue. While most commonly affecting the mediastinum and/or lungs, sarcoid can also affect the liver, resulting in "hepatic sarcoid." The spectrum of disease in hepatic sarcoid is variable, ranging from asymptomatic or mild liver enzyme abnormalities to end-stage liver disease requiring liver transplantation. Because clinically-significant hepatic sarcoid is rare, research on epidemiology, clinical manifestations, and treatment is limited. The mainstays of hepatic sarcoid treatment have involved steroids and more recently, ursodeoxycholic acid; however, novel immunomodulatory agents provide new possibilities for management. Questions remain regarding screening, diagnostic modalities, and what to treat with and when. The purpose of this review is to summarize the available research on the epidemiology, clinical course, and management of hepatic sarcoid, and identify gaps in our knowledge to motivate future research.
结节病是一种多系统炎症性疾病,其特征是在健康组织中出现非干酪样肉芽肿。虽然结节病最常影响纵隔和/或肺部,但也可能影响肝脏,导致“肝结节病”。肝结节病的疾病谱多种多样,从无症状或轻度肝酶异常到需要肝移植的终末期肝病。由于临床上显著的肝结节病很少见,因此对其流行病学、临床表现和治疗的研究有限。肝结节病的主要治疗方法包括类固醇和最近的熊去氧胆酸;然而,新型免疫调节剂为治疗提供了新的可能性。关于筛查、诊断方式以及用什么治疗以及何时治疗仍存在疑问。本综述的目的是总结肝结节病的流行病学、临床病程和治疗的现有研究,并确定我们知识中的差距,以推动未来的研究。