Clinical Features, Management and Prognosis of Hepatic Sarcoidosis: Insights From a Nationwide Italian Study

Sarcoidosis is a rare systemic granulomatous disease involving the liver in up to 20% of cases. Data on hepatic sarcoidosis (HS) prevalence and management remain limited. This study aims to provide a comprehensive analysis of HS patients across Italy, focusing on diagnostic pathways, management strategies, and prognostic factors.

This multicenter retrospective study, conducted from April 2022 to December 2023, includes data from 36 hepatology units, affiliated with the Italian Association for the Study of the Liver (AISF), invited to analyse consecutive cases of HS reported between 2003 and 2023.

A total of 78 patients with HS were identified, with complete data available for 58 (median age 53 years; 57% female; 81% Caucasian), prospectively followed for a median of 41 months. Pulmonary and lymphatic involvement were present in 45% and 34% of cases, respectively. Isolated hepatic involvement was seen in 10%. Liver biopsy revealed granulomas in all specimens. Histological cirrhosis and clinically significant portal hypertension (CSPH) were observed in 10% and 14% of patients, respectively. Patients with CSPH had a significantly higher body mass index (BMI) compared to those without (33 vs. 25, p = 0.002). Steroids and ursodeoxycholic acid were the first-line treatments for 80% and 28% of patients, respectively, while 29% required second-line therapies. One patient died from liver-related complications.

This nationwide study underscores the variability in HS presentation and management across Italy. Liver biopsy remains essential for diagnosis and staging. While steroids are the primary treatment, many patients require second-line therapies. Our finding of a higher BMI in patients with CSPH suggests that metabolic factors may play a role in disease progression, warranting further investigation.