Hepatic sarcoidosis:an Italian multicenter study

Introduction Sarcoidosis is a systemic granulomatous disease involving the liver in about 10-20% of cases. In absence of high-quality data on the management of hepatic sarcoidosis (HS), it is still experienced-based. Aim To collect and analyze data on the Italian real-world experience in HS management. Methods, Results Thirty-six AISF-affiliated hepatologic centers received questionnaires, thirty-three of them reported managing 78 patients with HS. Eighteen centers subsequently transmitted demographic and clinical data belonging to 11 Italian regions. Statistical analyses were performed with R Studio. 55 patients were included, median age at diagnosis was 52(44.8-57.3).Female patients were 33 (60%) and 45 (82%) patients were Caucasian.Most patients (45%) reported systemic symptoms (asthenia, fever) and a cholestatic pattern of liver enzymes elevation (Table1). Males had a significantly higher level of GGT (p 0.02) and bilirubin (p<0.01) at presentation. Figure1 shows radiological findings. In coexistence with HS, mainly pulmonary (43%) and lymphatic (36%) involvement were reported. Patients with only HS were 6 (10.9%).Clinically-significant-portal-hypertension (CSPH) at diagnosis was detected in 8(14.5%) patients according to LSM≥25Kpa or varices, only one of them had histological cirrhosis. BMI was significantly higher in patients with CSPH(p 0.01). Twenty-six patients were treated with first-line steroid therapy (48%), 11(20%) patients with steroid+ ursodeoxycholic-acid (UDCA), 6(11%) patients with UDCA only, 11 (20%) with combination of different agents, including anti-metabolites. Median alkaline-phosphatase (ALP) decrease was 56,3% in 12 months, independently from the medical treatment used (p>0.09). Seventeen (31%) patients received treatment adjustment with a second line agent. None of the patients underwent liver transplantation or developed hepatocellular carcinoma. One patient died during follow-up owing to liver-related complications. Conclusion HS is a rare entity with a heterogeneus clinical presentation and management. HS has an intrinsic risk of CSPH not only explained by the presence of cirrhosis, that is higher in patients with higher BMI; these findings need further investigations. Drug choice doesn't have an impact on ALP decrease, but UDCA are associated to a better GPT-reduction. A multicentric, prospective, national cohort study is eeded to further study this disease.