Paris, june 18-19, 2018

Campus des Cordeliers

Address: 15 rue de l’École de Médecine

75006 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)

Meeting location

Novotel Paris les Halles

Address: 8 Place Marguerite de Navarre

75001 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)

Accommodation

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

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

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

Chairs: Mario Strazzabosco, Yale & Michael Trauner, Vienna

Selected Proposals

Expected Outcomes

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.

Chairs: Christoph Schramm, Hamburg & Lionel Arrivé, Paris

Selected Proposals

Expected Outcomes

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.

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 (nelly.guitard@aphp.fr)