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ERKNet Meeting London 2 September 2019

Join the European Rare Kidney Disease Reference Network (ERKNet) meeting in London on 2 September 2019. Learn about ERKNet's vision of harmonizing disease management and promoting research in rare kidney diseases. Don't miss this opportunity to network with experts in the field.

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ERKNet Meeting London 2 September 2019

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  1. RaDaR Meeting, London 2 September 2019 ERKNet European Rare Kidney Disease Reference Network Tess Harris, PKD Charity E-PAG rep for ERKNet and Rare Liver ERN

  2. The ERN Vision: Everyone in Europe benefits from the same good healthcare pathways (diagnosis, prognosis, treatment)

  3. Background: the road to ERNs 2014 Commission Decisions on ERN 2013 EUCERD Recommendations European Reference Networks (RD ERNs) 2011 Directive application of patients' rights in cross-border healthcare Article 12 ERN 2006 RD Task Force Working Group on centres of reference Public Health Programme First pilot networks projects 2005 High Level Group on Health Services and Medical Care Centres of Reference report 2002 Free movementof patients

  4. The journey continued rapidly 2017 ERNs launched 2016 – Q3 Establishment of Networks 2016 – Q1-2 Call for Networks Proposals assessed 2015 Assessment manual Selection process 2014 Entry into force/legal acts

  5. March 2017

  6. ERKNetEuropean Rare Kidney Disease Reference Network Germany: Cologne Essen Hamburg Hannover Heidelberg Münster Sweden: Stockholm 43,000 patients UK: Birmingham London GOSH London RFH Manchester Newcastle Finland: Helsinki Lithuania: Vilnius 38 centres12 countries NL: Nijmegen Utrecht Amsterdam Poland: Warsaw Czechia: Prague 28 paediatric 22 adult Belgium: Brussels Leuven Italy: Siena Torino Milano Padova Naples I Naples II Florence Bergamo Rome Gemelli Rome Bambino Gesu France: Paris Necker Paris Mondor Paris Tenon Lyon Toulouse Spain: Barcelona

  7. ERKNet objectives Harmonizing disease management: Adoption and developmentof guidelines and pathways Monitoring performance and outcomes: Patient registries Promoting research and innovation Online disease information Virtual Consultation: Clinical PatientManagement System Training: CMEs, Webinars, eLearning

  8. ERKNet: Scope of Diseases Glomerulopathies Idiopathic NS SRNS/FSGS Alport Fabry MPGN Membranous GN Other immune-mediated GN CAKUT & Ciliopathies Syndromic Renal hypo/dysplasia ARPKD Nephronophthisis Bardet Biedl Syndrome PUV/obstructive uropathies AD renal dysplasia disorders ADPKD Tuberous sclerosis, VHL ADTKD (UMOD, HNF1ß, MUC1, REN, ..) Tubulopathies Renal tubular acidosis Bartter-like disorders Disorders of Mg homeostasis Disorders of Ca homeostasis Disorders of P homeostasis Dent‘s disease Lowe syndrome Metabolic and stone forming disorders Primary hyperoxaluria Cystinosis Cystinuria MMA Thrombotic microangiopathies Pediatric CKD stage 3-5 and Dialysis Pediatric transplantation

  9. Patient numbers by disease Total n=43,135

  10. ERKNetEuropean Rare Kidney Disease Reference Network Coordinator Network Secretariat Related ERNs Executive Board (Coordinator, 1 representative per workgroup, ePAG) ePAG Thematic Work Groups Task Forces ERA-EDTA WGiKD Hereditary glomerulo-pathies Thrombotic microangio-pathies Molecular diagnostics Pathology & Imaging Immune glomerulo-pathies Transition & QoL Registries & Outcomes CAKUT & Ciliopathies AD dysplasias ESPN work groups Obstructive uropathies Education & Training Guidelines & Pathways Metabolic nephropathies Tubulopathies ERA-EDTA EURBP Pediatric CKD& Dialysis Pediatric Tx Research KDIGO Network Board (1 representative per member)

  11. European Rare Kidney Disease Registry • Core Registry for all rare kidney diseases • Objectives: • To identify current patient cohorts for clinical research • Performance assessment, benchmarking: Disease-specific key quality and outcome indicators Open to all European nephrology centers interested in rare diseases Option to incorporate disease-specific sub-registries: ESPN dRTA Registry Funding:Kickoff grant: WGIKD 2017/18Operational grant: EU-Chafea 2018-2020

  12. Registry structure Local/ regional/ national patient registries Centers Direct data entry • Center administration • Disease demographics • Trial cohort information • Monitoring of guideline adherence, performance and outcome Indicators ERKNet Registry RaDiCo Cystinosis RaDiCo Alport EURenOmics Membranous Nephropathy PodoNet SRNS NeoCyst Cilio-pathies AReg PKD ADPedKD IPDN Pediatric Dialysis CERTAIN Pediatric Transplant • Genotype-phenotype information • Natural history information • Best medical practice information

  13. ERN-Wide Data Collection • Common data set including - the JRC minimum data set, ensuring full inter-operability with other RD registries - essential information relevant to all patients with rare kidney diseases • Disease-specific data set 40 key performance and outcome indicators (KPI) defined by thematic workgroups in Delphi consensus process ERKRegcollectstwo datasets: • Disease specific sub-registries: • Modular approach allows extended data collection for diseases of interest • ESPN distal RTA registry • Italian Alport registry • Current requests: Cystinuria, Tubulointerstitial nephritis, ADTKD Under development: Automated data transfer from hospital electronic health records

  14. Enrolment Status ERKReg The Rare Renal Registry

  15. ERKNet Current numbers

  16. 69 disease causing genes in 375/1298 registered cases (29%),375/505 hereditary disease cases (74%)

  17. ERKNet Performance and outcome monitoring

  18. ERKNet ERKNet: Patient engagement • Representing patients in ERN governance • Voting members of ERKNet Board • Involved in all disease specific working groups and transversal groups • Aligned with ERKNet scope

  19. ERKNetEuropean Rare Kidney Disease Reference Network EPAGs involved across ERKNet Coordinator Network Secretariat Related ERNs Executive Board (Coordinator, 1 representative per workgroup, ePAG) ePAG Thematic Work Groups Task Forces ERA-EDTA WGiKD Hereditary glomerulo-pathies Thrombotic microangio-pathies Molecular diagnostics Pathology & Imaging Immune glomerulo-pathies Transition & QoL Registries & Outcomes CAKUT & Ciliopathies AD dysplasias ESPN work groups Obstructive uropathies Education & Training Guidelines & Pathways Metabolic nephropathies Tubulopathies ERA-EDTA EURBP Pediatric CKD& Dialysis Pediatric Tx Research KDIGO Network Board (1 representative per member)

  20. Project: ‚Kidney Patient Journeys‘ • Developed by patients (ePAGs) • Medical details approved by ERN professionals • Published online • Contents: • Typical patient pathway • Typical challenges and pitfalls • Medical issues where specialists can help • Unmet patient needs at various disease stages

  21. ERKNet ERKNet: Future Current ERKNet membership: 38 healthcare providers 32 pediatric, 18 adult units 2nd call 07/2019: 13+ centers to apply for full Membership 8+ centers to apply for Affiliated Partnership -> Coverage to increase from 12 to 22 EU countries • Brexit: • UK centres – 3rd country status

  22. ERKNet • Special thanks to Prof. Dr. Franz Schaefer • ERKNet Coordinator • Visit ERKNet website for latest info https://www.erknet.org/index.php?id=home

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