International PSC Study Group meeting Oslo, 14th – 15th June 2010

1. International PSC Study Group meetingOslo, 14th – 15th June 2010 International PSC database – parameter consensus Kirsten Muri Boberg, Oslo Tobias Weismüller, Hannover

2. International PSC database: Agenda • Purpose • Type/structure of database (3min-Talk of C. Ponsioen and of C. Bowlus) • Organization of database • Responsibilities • Legal aspects/personal data protection • Costs • Parameters (3min-Talk of C. Schramm) 2

3. International PSC database: Purpose • Provide key data for international collaborations • Identification of specific subgroups for further studies • CCA, small-duct etc. • DNA collections and genetic studies • Epidemiologic studies • Identification of clinical risk factors • Other? 3

4. International PSC database: Purpose Discussion 4

5. International PSC database: Type & Structure • Should be constructed for robustness, durability and flexibility • Upgrading should be possible • Avoid solutions like Access • Accessable to all centers • One database, but automatic access only to own data (password) • Terminal server or web-based • Technology for access to the database must be available at each center • Experiences? • Local databases • German HEPNET-database • Nordic Liver Transplant Registry • Others? 5

6. International PSC database: Type & Structure 3 min Talk of C. Ponsioen: Dataset of the Epi PSC/PBC study 6

7. International PSC database: Type & Structure 3 min Talk of C. Bowlus: Online PSC-research network application 7

8. International PSC database: Type & Structure Discussion 8

9. International PSC database: Organisation • Where will the database be located? • Link to local databases and biomaterials? • (ID-number, see later) • Who decides which data will be included? • Agreement on some data during this meeting • Additional suggestions can be co-ordinated and sent out for a vote (accept if ≥50% agree?) • Who decides on data access for specific projects? 9

10. Centre A/B/C/D/E or PSC Study Group suggests a project to all centres • Centres that agree, participate Data of Patient A-12345 Data of Patient E-12345 Data of Patient B-12345 Data of Patient C-12345 International PSC database: Organisation After approval from centre A/B/C/D, the centre taking the initiative to a study must contact the database co-ordinator to obtain the data 10

11. International PSC database: Responsibilities • Co-ordination and follow-up • Located to one center • Co-ordinator has access to all data • Access from all centers to own data only • Responsibility for completeness and update • Each center • Responsibility for the whole database • Steering committee? 11

12. International PSC database: Organisation and Responsibilities Discussion 12

13. International PSC database: Legal aspects • Legal aspects must be cleared by each center • Patients`consent • Ethical committee • Data processing • Personal data protection • Identity of patient only known to the recruiting centre • Identification through an individual ID-Number (centre-code + patient code) (what is allowed?) • Secure internet connection 13

14. International PSC database: Legal aspects Discussion 14

15. International PSC database: Costs • Non-recurring costs • Development of database • Hardware (server, computer) • Current costs • IT-technician • Study nurse • Co-ordinator 15

16. International PSC database: Costs Discussion 16

17. International PSC database: Parameters • Aim for completeness of the parameters selected • Focus on a limited number (30? 40? 50?) • Local extensions or connections to other databases should be the responsibility of each center 17

18. 18

19. Liver disease Date first symptom Date first biochemical abn. Date diagnosis liver disease Cholangiographical findings Type of cholangiography Malignancy (CCA, HCC etc) Date diagnosis malignancy Revised diagnosis liver Date revised diagnosis IBD Date first symptom Date diagnosis IBD Type of IBD Date colectomy Cause of colectomy Malignancy Date diagnosis malignancy Endpoints Date OLT 1, 2, 3 Date of death Cause of death Hepatitis serology Smoking (at time of diagn.) Alcohol (at time of diagn.) Other autoimmune disorder in the patient Gall stone Comments Main parameters in the Norwegian PSC biobank database 19

20. Liver disease Date first symptom Date first biochemical abn. Date diagnosis liver disease Cholangiographical findings Type of cholangiography Malignancy (CCA, HCC etc) Date diagnosis malignancy Revised diagnosis liver Date revised diagnosis IBD Date first symptom Date diagnosis IBD Type of IBD Date colectomy Cause of colectomy Malignancy Date diagnosis malignancy Endpoints Date OLT 1, 2, 3 Date of death Cause of death Hepatitis serology Smoking (at time of diagn.) Alcohol (at time of diagn.) Other autoimmune disorder in the patient Gall stone Comments Parameters: further discussion 20

21. International PSC database: Type & Structure 3 min Talk of C. Schramm: additional parameters: pregnancy in PSC 21

22. International PSC database: Parameters • Liver disease • Date first symptom • Date first biochemical abn. • Date diagnosis liver disease • Cholangiographical findings • Type of cholangiography • Malignancy (CCA, HCC etc) • Date diagnosis malignancy • Revised diagnosis liver • Date revised diagnosis • IBD • Date first symptom • Date diagnosis IBD • Type of IBD • Date colectomy • Cause of colectomy • Malignancy • Date diagnosis malignancy • Endpoints • Date OLT 1, 2, 3 • Date of death • Cause of death • Hepatitis serology • Smoking (at time of diagn.) • Alcohol (at time of diagn.) • Other autoimmune disorder in the patient • Gall stone • Comments Discussion 22

23. 23

24. Main diagnosis liver disease PSC Small duct PSC PSC/AIH ”overlap” IgG4 Date diagnosis liver disease PSC: date first cholangiography compatible with PSC Small duct PSC: date when collective findings give this conclusion (biopsy, cholangiography) Findings at cholangiography Normal IH + EH-PSC IH-PSC (without EH-PSC) EH-PSC (without IH-PSC) Hepatobiliary malignancy(confirmed by histology or cytology) None CCA CCA in situ (BilIN3) (in explanted liver) Gallbladder carcinoma HCC Pancreatic carcinoma Other cancer in the liver region (not classified within the above groups) Unknown 24

25. Date diagnosis IBD Date colonoscopy with macroscopic and/or microscopic findings compatible with IBD Type of IBD None UC CD IBD unclassified Unknown Extension of IBD (macro/micro) Left-sided (distal of left flexure) Right-sided (prox. of left flexure) Total colitis Proctitis Pouchitis Cause of colectomy Active disease Dysplasia (low-grade) Dysplasia (high-grade/ca. in situ) DALM (dysplasia associated lesion or mass) Colon carcinoma Malignancy, bowel None Dysplasia (low-grade) Dysplasia (high-grade/ca. in situ) DALM Carcinoma Unknown 25

26. Date OLT (no. 1, 2, 3..) Recurrence after OLT Date of death Cause of death Liver failure Hepatopancreaticobiliary cancer Colon cancer Other cancer Other cause Smoking history (recorded at time of diagnosis liver disease) Yes – current smoker (regular use) Ex-smoker Never smoker Unknown Alcohol (recorded at time of diagnosis liver disease) (units/d) 0 – 1 >1 – 3 >3 Unknown Autoimmune disease in patient DM type 1 Thyroid disease Coeliac disease Reumatoid arthritis Sarcoidosis Psoriatic disease Nephritis Vitiligo SLE Vasculitis Fibrosing alveolitis Sacroileitis 26