The ERKReg registry is not only a vital tool for clinical documentation and quality monitoring—it also serves as a valuable resource for collaborative research. Over the past years, numerous scientific projects have been initiated based on ERKReg data, resulting in a growing number of peer-reviewed publications that contribute to the understanding and management of rare kidney diseases.

If you are an investigator interested in conducting your own analysis using ERKReg data, please visit our Data Access Policy section for detailed information on how to submit a research proposal and the procedures for accessing the dataset.

For further inquiries, feel free to contact us at erkreg@erknet.org.

1) Methodology Paper of the European Rare Kidney Diseases Registry (ERKReg)

The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results. Bassanese et al. Orphanet J Rare Dis (2021)

2) Comparison of childhood ADPKD data from ERKReg with other major registries

Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidneydisease in high- and middle-income countries, Gimpel et al., Kidney International 

3) Prescription patterns and outcomes of C5 Inhibitor Therapy in aHUS

Real-world use of complement inhibitors for haemolytic uraemic syndrome: an analysisof the European Rare Kidney Disease Registry cohort. Vujović et al., eClinicalMedicine, Volume 82, 103159

• Characterization of clinical courses of patients with kidney a/hypo/dysplasia (Dziwisz, Kohl, Liebau)

  Status: Data analysis is ongoing; preliminary results were presented at the ERKNet meeting in Leuven, and publication is planned once analysis is completed.

• Genetic variant Interpretation and validation of patients with genetic diseases (Cozzi, vanEerde, Van der Velde)

  Status: Currently finalizing the analyses of over 8000 genetically tested patients.

• Observational study on epidemiological data in STEC-HUS patients in ERKReg (Soto, Van de Kar)

  Status: Data analysis is ongoing; results extracted from ERKReg were presented at the ERKNet Annual Meeting and in the TMA Working Group session in Leuven. A complementary questionnaire has been developed for more detailed information. The data have not yet been published, and a manuscript is currently in preparation.

• Presentation and early outcome in juvenile SLE nephritis (Suhlrie, Vivarelli)
  
  Status: Data overview is still in progress; analysis and publication are planned once completed. 

• Characterization of patients carrying SLC4A4 variants causing proximal RTA (Secondulfo, Petzold)
  
  Status: Data has been extracted from ERKReg; a complementary survey is in preparation. A manuscript is planned for the end of 2025 with the intention to publish.

   

• Adult outcome of childhood SSNS (Servais, Bassanese)
• Clinical practice and genetic screening in glomerulopathies (Roccatello, Bassanese)
• Causes and consequences of long-term dialysis of children (Bacchetta, Ranchin)
• Prediction tool for eGFR decline in NPHP1-related nephronophthisis (König)

• Roche: Demographic analysis of aHUS -→ finalised
• Vertex: APOL-L1 mediated kidney disease and ADPKD -→ finalised
• Novartis: Disease characteristics and treatment patterns of C3G/IC-MPGN -→ finalised
• Roche: Treatment Patterns for Membranous Nephropathy in ERKReg -→ finalised
• NovoNordisk & OxalEurope : A comparison of clinical characteristics, diagnosis and outcomes in rare kidney stone forming diseases -→ ongoing
• STADA Pharma: Diease characteristicis and treatment patterns of IgANephropathy -→ ongoing