Hepatic sarcoidosis: Clinical characteristics and outcome.

Autor:	Graf C; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Arncken J; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Lange CM; Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany., Willuweit K; Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany., Schattenberg JM; Department of Internal Medicine I, University Medical Center Mainz, Mainz, Germany., Seessle J; Department of Internal Medicine IV, University of Heidelberg, Heidelberg, Germany., Lang-Meli J; Department of Medicine II, University Hospital Freiburg, Freiburg, Germany., Böttler T; Department of Medicine II, University Hospital Freiburg, Freiburg, Germany., Dietz J; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Wetzstein N; Department of Infectious Diseases, University Hospital Frankfurt, Frankfurt, Germany., Mondorf A; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Vermehren J; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Rohde G; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Zeuzem S; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany., Pathil A; Department of Internal Medicine I, University Hospital Frankfurt, Frankfurt am Main, Germany.
Jazyk:	angličtina
Zdroj:	JHEP reports : innovation in hepatology [JHEP Rep] 2021 Sep 03; Vol. 3 (6), pp. 100360. Date of Electronic Publication: 2021 Sep 03 (Print Publication: 2021).
DOI:	10.1016/j.jhepr.2021.100360
Abstrakt:	Background & Aims: 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. Lay Summary: 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. Competing Interests: CG has received travel support from Gilead and speaking fees from AbbVie outside the submitted work. JMS is a consultant at BMS, Boehringer Ingelheim, Echosens, Genfit, Gilead Sciences, Intercept Pharmaceuticals, Madrigal, Nordic Bioscience, Novartis, Pfizer, Roche, Sanofi, and Siemens Healthcare GmbH. JMS also has received research funding from Gilead Sciences, Boehringer Ingelheim, and Siemens Healthcare GmbH. JMS has received speakers’ honoraria from Falk Foundation and MSD Sharp & Dohme GmbH all outside of the submitted work. CML has received speaker and consultancy fees outside the present work from AbbVie, Gilead, Falk, MSD, Novartis, Roche, and Eisai. JD has received research support from Gilead outside the submitted work. JV has received speaking and/or consulting fees from Abbott, AbbVie, Bristol-Myers Squibb, Gilead, Medtronic, Merck/MSD, and Roche outside the submitted work. SZ has received speaking and/or consulting fees from AbbVie, Bristol-Myers Squibb, Falk, Gilead, Janssen, and Merck/MSD outside the submitted work. AP has received travel support from AbbVie and BMS and lecturer fees from AbbVie, BMS, and Gilead. All other authors declare no competing interests. Please refer to the accompanying ICMJE disclosure forms for further details. (© 2021 The Authors.)
Databáze:	MEDLINE
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