Autoimmune disease of any form means an individual’s own immune system interprets normal body tissue to be foreign. The result is that the immune system attacks the tissue it views as hostile. In Autoimmune Hepatitis (AIH) the immune system attacks the liver cells called hepatocytes. In Primary Biliary Cholangitis (PBC) and Primary Sclerosing Cholangitis (PSC) the immune system attacks the bile duct cells, called cholangiocytes. These diseases may be evolutive and can led to cirrhosis and its complications (liver failure and malignancies). Wilson disease (WD) is a rare autosomal recessive genetic disease of copper metabolism that results in pathologic deposition of copper in the liver, brain, and other organs and tissues that is fatal, if left untreated.

Through our research, we hope to better understand the pathogenesis, the subtypes of these disease, their natural history and to test new treatments. Our patients have the opportunity to be enrolled into national and international disease registry and participate in several clinical trials for treatment of disease and symptoms.

Publications

1. Safety and efficacy of elafibranor in primary sclerosing cholangitis: The ELMWOOD phase II randomized-controlled trial. J Hepatol. 2025 May 8: S0168-8278(25)00252-1.
2. Cirrhosis and age are key determinants of HCC risk in individuals with primary sclerosing cholangitis: A multicenter longitudinal cohort study. Hepatology. 2025 Apr 16. doi: 10.1097/HEP.0000000000001351.
3. Development of a question prompt list for people living with primary biliary cholangitis - A Delphi Study. Clin Gastroenterol Hepatol. 2025 Apr 15:S1542-3565(25)00293-9.
4. Position paper of the Italian Association for the Study of the Liver (AISF): Management and treatment of primary biliary cholangitis. Dig Liver Dis. 2024 Sep;56(9):1461-1474.
5. Prognostic scores for ursodeoxycholic acid-treated patients predict graft loss and mortality in recurrent primary biliary cholangitis after liver transplantation. J Hepatol. 2024 Oct;81(4):679-689.
6. Development and Validation of a Scoring System to Predict Response to Obeticholic Acid in Primary Biliary Cholangitis. Clin Gastroenterol Hepatol. 2024 Oct;22(10):2062-2074.e11
7. Quantitative magnetic resonance cholangiopancreatography metrics are associated with disease severity and outcomes in people with primary sclerosing cholangitis. JHEP Reports 2022; Sep 3;4(11):100577
8. Cholangiocyte senescence in primary sclerosing cholangitis is associated with disease severity and prognosis. JHEP Reports. 2021;3:100286.
9. The Complementary Value of Magnetic Resonance Imaging and Vibration-Controlled Transient Elastography for Risk Stratification in Primary Sclerosing Cholangitis. Am J Gastroenterol 2019;114: 1878-1885.
10. EASL Clinical Practice Guidelines on non-invasive tests for evaluation of liver disease severity and prognosis – 2021 update. J Hepatol. 2021;75:659-689.

Funding

Assegno SID 2022
EU Horizon 2020, European Joint Programme for Rare Disease 2023

Clinical Trials

- A Phase III Randomised, Parallel-Group, Double-Blind, Placebo-Controlled, Two-Arm Study to Evaluate the Efficacy and Safety of Elafibranor 80 mg on Long-Term Clinical Outcomes in Adult Participants with Primary Biliary Cholangitis (PBC). Eudract:2023-505251-43-00
- A Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Volixibat in the Treatment of Cholestatic Pruritus in Patients with Primary Biliary Cholangitis.  Eudract: 2021-001389-39.
- A Randomized Double-Blind Placebo-Controlled Study to Evaluate the Efficacy and Safety of Volixibat in the Treatment of Cholestatic Pruritus in Patients with Primary Sclerosing Cholangitis. Eudract : 2020-003027-41.
- A Randomized, Double-Blind, Placebo-Controlled, Study to Evaluate the Effect of Seladelpar on Clinical Outcomes in Patients with Primary Biliary Cholangitis (PBC) and Compensated Cirrhosis. Eudract:2024-516525-31-00.
- A Phase II, Multicenter, Double-Blind, Randomised, Placebo-Controlled Study and Open- Label Long Term Extension to Evaluate the Safety and Efficacy of Elafibranor in Adult Participants with Primary Sclerosing Cholangitis (PSC). 
- An Open Label, Phase 2 Study to Evaluate the Effect of A3907 on Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics in Adults with Primary Sclerosing Cholangitis (PSC).
- Double-blind, Randomized, Placebo-controlled, Phase III Study Comparing norUrsodeoxycholic Acid Capsules With Placebo in the Treatment of Primary Sclerosing Cholangitis
- A randomized, double-blind, multi-dose, placebo-controlled study to evaluate the efficacy, safety and tolerability of GSK2330672 administration for the treatment of pruritus in participants with primary biliary cholangitis.
- Studio prospettico sul valore prognostico dell’elastomeria epatica (fibroscan) nei pazienti con colangite sclerosante primitiva (FICUS study).
- Registro Nazionale Colangite Biliare Primitiva. Registro PBC 322
- European Reference Network “RARE-LIVER”

People involved

Nora Cazzagon, Assistant Professor
Elisa Catanzaro, PhD student
Francesco Paolo Russo, Associate Professor
Raffaella Motta, Associate Professor (DIMED)

Group members

Emanuela Bonaiuto (Post Doc), Marco Senzolo, (Consultant Transplant Hepatologist), Martina Gambato (Consultant Transplant Hepatologist),  Martina Benatti, Giovanni Figorilli, Matteo Peviani (GI Fellows)