Paris, june 18-19, 2018

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1 Paris, june 18-19, 2018

2 Meeting location Campus des Cordeliers Address: 15 rue de l École de Médecine Paris France Metro: Line 4 or 10, Odéon Station RER (city trains): Line B, Saint-Michel or Luxembourg station Line C, Saint-Michel station Bus: 38, 27, 58 (Stops: Rue des Écoles or Odéon)

3 Accommodation Novotel Paris les Halles Address: 8 Place Marguerite de Navarre Paris France Metro: Lines 4, 7 and 11, get off at the Châtelet station and take the Place Saint Opportune exit. On metro lines 1 and 14, take the Rue de Rivoli exit. On RER lines A, B and D, take the Forum des Halles exit then Rue Berger. RER (city trains): Lines A, B and D, get off at Châtelet-Les Halles station and take the Forum des Halles exit then Rue Berger. Bus: 38 (stop: Châtelet) and 27 (stop: Pont neuf- Louvre)

4

5 Monday 18 June: 11:30 AM : Lunch Buffet 12:50 PM : Welcome Olivier Chazouillères, Paris 1:00 PM: Disease staging Olivier Chazouillères & Annarosa Floreani, Padova Serum tests Cyriel Ponsioen, Amsterdam Histology Stefan Hubscher, Birmingham Elastography Christophe Corpechot, Paris 2:30 PM Gut-liver axis Chantal Housset, Paris & Johannes Espolin Roksund Hov, Oslo The microbiome and the gut-liver axis, Harry Sokol, Paris Impact of liver and colon diseases on each other, Annika Bergquist, Stockholm Immunology of the gut-liver axis, Evaggelia Liaskou, Birmingham 4:00 PM: Coffee Break 4:30 PM: Working groups Organoids models and biobanking, Mario Strazzabosco, Yale & Michael Trauner, Vienna Natural history and Database, Cyriel Ponsioen, Amsterdam & Christopher Bowlus, UC Davis Tuesday 19 June: 8 :30 AM: Treatment Gideon Hirschfield, Birmingham & David Assis, Yale Clinical trials, Cynthia Levy, Miami Bile acid targeting, Emina Halibasic, Vienna Immune targeting Johannes Herkel, Hamburg Fibrosis targeting, Yuri Popov, Boston 10:30 AM: Coffee break 11:00 AM Working group leaders reports & unmet needs Ulrich Beuers, Amsterdam & Tom Karlsen, Oslo Organoids models and biobanking Natural history and Database Imaging studies Malignancies in PSC ERN Ansgar Lohse, Hamburg & Albert Pares, Barcelona Working groups format Ulrich Beuers, Amsterdam & Tom Karlsen, Oslo 12:30 AM Concluding remarks Chantal Housset & Olivier Chazouillères, Paris 12:45 AM 2:30 PM : Lunch Buffet Imaging studies, Christoph Schramm, Hamburg & Lionel Arrivé, Paris Malignancies in PSC Luca Fabris, Padova & Jesus Banales, San Sebastian 6:00 PM: Patients Associations (Albi, PSC Europe, PSC Partners) and Fonds CSP 8:00 PM: Dinner

6 Chairs: Mario Strazzabosco, Yale & Michael Trauner, Vienna Selected Proposals Expected Outcomes

7 Chairs: Cyriel Ponsioen, Amsterdam & Christopher Bowlus, UC Davis During the 1.5 hours working group session on Natural History & Database we welcome proposals on mining the existing IPSCSG retrospective database for observational studies. Alternatively, joint ventures between several centers are also welcomed. Participants are asked to present their proposals in a 3-4 slides presentation. Also, we invite attendees to participate in discussions on a uniform prospective IPSCSG database, tentatively coined the IPSCR.

8 Chairs: Christoph Schramm, Hamburg & Lionel Arrivé, Paris Selected Proposals Expected Outcomes

9 Chairs: Jesus M. Banales & Luca Fabris Selected Proposals Whole exome sequencing of PSC-associated biliary tract cancers (Trine Folseraas, Oslo) CCA Registry database (update: Jesus M. Banales, San Sebastian) Animal models of PSC-CCA (update: Luca Fabris, Padua) International validation study of new non-invasive protein biomarkers inserum extracellular vesicles of PSC, CCA, HCC patients (Jesus M. Banales, San Sebastian) Imaging of peribiliary glands and histo-morphology correlates in PSC-CCA. A multicentre international study proposal (V. Cardinale, G. Carpino, Rome) Metabolomics and lipidomics in assessment of disease progression and risk for biliary dysplasia in PSC (M. Farkkila, Helsinki) PSC to CCA - Which role does the stroma play? (J. Mertens, Zurich) Role of PSC-related intestinal dysbiosis in colitis-associated colorectal cancer (Massimo C. Fantini, Rome) Expected Outcomes This workshop will propose international collaborative studies for participants interested in the search of non-invasive biomarkers for early diagnosis of CCA in PSC patients, and on the prediction of CCA development, using innovative methods, including molecular, histological and radiological approaches. Moreover, novel collaborative projects on the association between genetics and PSC-associated biliary cancer will be presented. Finally, collaborative proposals on new animal models for the study of PSC-CCA, novel strategies for the analysis of the role of stroma in the progression of PSC to CCA, and on the microbiome in PSC-related colorectal cancer will be outlined.

10 Olivier Chazouillères, Christophe Corpechot and Chantal Housset Sorbonne Université & Inserm, Saint-Antoine Research Center (CRSA) Assistance Publique-Hôpitaux de Paris (AP-HP), Saint-Antoine Hospital, Hepatology Department, Reference Center for Inflammatory Biliary diseases and Auto-immune Hepatitis Nelly Guitard and Virginie Tsilibaris FILFOIE (French Network for rare liver disease). Contact: Nelly Guitard

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