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伪装成长期胆汁淤积的结节病

Sarcoidosis Masquerading as Long-Standing Cholestasis.

作者信息

Deliwala Smit S, Hussain Murtaza, Ponnapalli Anoosha, Khanal Rupesh, Goyal Hemant, Abdalla Adil, Elbedawi Mamoon M

机构信息

Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA.

Division of Gastroenterology, Department of Internal Medicine, The Wright Center for Graduate Medical Education, Scranton, PA, USA.

出版信息

Gastroenterology Res. 2021 Apr;14(2):112-115. doi: 10.14740/gr1360. Epub 2021 Apr 21.

Abstract

Hepatic sarcoidosis is an exceedingly rare extrapulmonary manifestation of sarcoidosis, with the majority remaining stable for years without clinical clues, only displaying biochemical abnormalities. Amongst the literature, the timeline to cirrhosis has not been parsed out; hepatomegaly develops in 50% and cirrhosis in 33% of all hepatic sarcoidosis patients, making this an essential issue in this patient population. Interestingly, the risk for hepatocellular carcinoma remains high regardless of cirrhosis development. Corticosteroids and biologics remain the mainstay of therapy, although refractory cases may require deeper immunosuppression. Liver transplantation is seen in a handful of cases with promising results. We present an interesting case of cholestatic pattern livery injury in our outpatient setting that was eventually discovered to be hepatic sarcoidosis. Mild biochemical derangements or sole elevations in alkaline phosphatase are under-recognized, and patients often progress to cirrhosis and end-stage liver disease. This diagnostic miss has significant implications and represents an opportunity to treat liver disease with a reversible cause. Consensus guidelines recommend alkaline phosphatase screening in newly diagnosed cases of sarcoidosis.

摘要

肝结节病是结节病极为罕见的肺外表现,大多数患者多年来病情稳定,无临床症状,仅表现为生化指标异常。在相关文献中,尚未明确肝结节病发展为肝硬化的时间线;在所有肝结节病患者中,50%会出现肝肿大,33%会发展为肝硬化,这在该患者群体中是一个关键问题。有趣的是,无论是否发展为肝硬化,肝细胞癌的风险仍然很高。皮质类固醇和生物制剂仍然是主要的治疗手段,尽管难治性病例可能需要更强的免疫抑制治疗。少数病例会接受肝移植,效果良好。我们在门诊遇到一例有趣的胆汁淤积型肝损伤病例,最终被诊断为肝结节病。轻度生化指标紊乱或单纯碱性磷酸酶升高往往未得到充分认识,患者常发展为肝硬化和终末期肝病。这种诊断失误具有重大影响,也意味着有机会治疗具有可逆病因的肝病。共识指南建议对新诊断的结节病病例进行碱性磷酸酶筛查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a05b/8110232/07213d3a8c85/gr-14-112-g001.jpg

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