Hepatic sarcoidosis: Clinical characteristics and outcome

• Published in: JHEP reports Vol. 3; no. 6; p. 100360
• Main Authors: Graf, Christiana, Arncken, Jeannette, Lange, Christian M., Willuweit, Katharina, Schattenberg, Jörn M., Seessle, Jessica, Lang-Meli, Julia, Böttler, Tobias, Dietz, Julia, Wetzstein, Nils, Mondorf, Antonia, Vermehren, Johannes, Rohde, Gernot, Zeuzem, Stefan, Pathil, Anita
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.12.2021; Elsevier
• Subjects: Hepatic granuloma; Hepatic sarcoidosis; Liver involvement; Outcome; Treatment; ATS; SBP; ACLF; AST; ACE; ICD-10; ALP; MMF; AZA; HRS; ALT; HCC; UDCA; Hepatic granuloma; IL-2R; MTX; Treatment; GGT; Outcome; ULN; Liver involvement; HE; Hepatic sarcoidosis; HE, hepatic encephalopathy; ACE, angiotensin-converting enzyme; SBP, spontaneous bacterial peritonitis; ALP, alkaline phosphatase; AZA, azathioprine; ICD-10, International Classification of Diseases, Tenth Revision; AST, aspartate transaminase; IL-2R, IL-2 receptor; ALT, alanine transaminase; MMF, mycophenolatmofetil; ACLF, acute-on-chronic liver failure; MTX, methotrexate; ULN, upper limit of normal; HRS, hepatorenal syndrome; HCC, hepatocellular carcinoma; UDCA, ursodeoxycholic acid; ATS, American Thoracic Society; GGT, gamma glutamyl transferase
• ISSN: 2589-5559; 2589-5559
• DOI: 10.1016/j.jhepr.2021.100360

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. 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. 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. 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. 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. [Display omitted] •There has been little research on clinical characteristics and the clinical outcome of patients with hepatic sarcoidosis.•One-third of patients with hepatic sarcoidosis presented with clinically significant portal hypertension, and 14.5% suffered from cirrhosis.•Biochemically, a cholestatic pattern of liver enzyme elevations was the most common abnormality.•The correct and early identification of hepatic involvement in sarcoidosis is crucial because of the potentially progressive course of disease.