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. Cazzagon N., Lemoinne S., El Mouhadi S. Trivedi, PJ, Gaouar F, Kemgang A.et al.  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.
2. Cazzagon N, Sarcognato S, Floreani A, Corrà G, De Martin S, Guzzardo V, Russo FP, Guido M. Cholangiocyte senescence in primary sclerosing cholangitis is associated with disease severity and prognosis. JHEP Rep. 2021;3:100286.
3. EASL Berzigotti A, Tsochatzis E, Boursier J, Castera L, Cazzagon N, Fiedrich-Rust M, Petta S, Thiele M. EASL Clinical Practice Guidelines on non-invasive tests for evaluation of liver disease severity and prognosis – 2021 update. J Hepatol. 2021;75:659-689.
4. Cazzagon N, El Mohuadi S, Vanderbecq, Ferreira C, Finnegan S, Lemoinne S et al. Quantitative magnetic resonance cholangiopancreatography metrics are associated with disease severity and outcomes in people with primary sclerosing cholangitis. JHEP Reports 2022;DOI:  https://doi.org/10.1016/j.jhepr.2022.100577
5. Murillo Perez CF, … Cazzagon N, Floreani A, Mason AL, Parés A et al. Greater Transplant-free Survival in Patients Receiving Obeticholic Acid for Primary Biliary Cholangitis in a Clinical Trial Setting Compared to Real-World External Controls. Gastroenterology 2022:S0016-5085(22)01060-5. doi: 10.1053/j.gastro.2022.08.054
6. Corpechot C, Carrat F, Gaouar F, Chau F, Hirschfield G, Gulamhusein A, Montano-Loza AJ, Lytvyak E, Schramm C, Pares A, Olivas I, Eaton JE, Osman KT, Dalekos G, Gatselis N, Nevens F, Cazzagon N, …, Global & ERN Rare-Liver PBC Study Groups. Liver stiffness measurement by vibration-controlled transient elastography improves outcome prediction in primary biliary cholangitis. J Hepatol. 2022 Jun 28:S0168-8278(22)00411-1. doi: 10.1016/j.jhep.2022.06.017.

Funding

Assegno SID 2022

Clinical Trials

- A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study Evaluating the Safety, Tolerability, and Efficacy of Cilofexor in Non-Cirrhotic Subjects With Primary Sclerosing Cholangitis.
- 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.
- Double-blind, randomized, placebo-controlled, phase II dose-finding study comparing different doses of RhuDex granules with placebo in the treatment of primary biliary cholangitis
- Studio osservazionale prospettico sull’efficacia e la sicurezza dell’acido obeticolico in pazienti affetti da colangite biliare primitiva non-responder alla terapia con acido ursodesossicolico (UDCA).
- 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

People involved

Nora Cazzagon, Assistant Professor
Francesco Paolo Russo, Associate Professor
Francesco D’Amico, Associate Professor
Raffaella Motta, Associate Professor (DIMED)

Group members

Emanuela Bonaiuto (Post Doc), Marco Senzolo, (Consultant Transplant Hepatologist), Martina Gambato (Consultant Transplant Hepatologist), Francesco Ferrara (Consultant Gastroenterologist)
Francesco Pezzato, Matteo Peviani, Erica D’Ovidio, Elisa Catanzaro (GI Fellows)