What is the Registry?

ERKReg is the first and largest Europe-wide registry for all rare kidney diseases. 

ERKReg collects prospective information from all patients with rare kidney diseases within the network who agree to have their de-identified data recorded.

The registry helps clinicians, researchers and pharmaceutical industry to generate more knowledge about rare kidney diseases and focus their research and drug development to improve the quality of life of our patients. The registry also aims to help patient organizations, health authorities and policy makers in optimizing the support for patients with rare kidney disorders.  

ERKReg is currently receiving patient data from more than 70 hospitals in 24 countries. 

Every month 300-400 new patients are entered to the ERKReg Registry.

Aims of the Registry

  • Demographic Analysis

    The ERKReg registry collects data from 24 countries and allows to analyse the frequency and characteristics of more than 300 rare kidney diseases in different regions of Europe.

  • Natural Disease History

    Patient information is collected once a year by the medical teams to follow the clinical course over time. This will help to identify risk factors for complications and disease progression.

  • New Therapies

    ERKReg data facilitates multicentre research projects to explore novel treatment strategies and new drugs that can improve the patient’s wellbeing and quality of life.

    The registry allows to find patients with a particular disease for which a new treatment is being developed. The registry team brokers the matching of patients with upcoming clinical trial opportunities.

    In this way, the registry supports the rapid access of our patients to new therapies.

  • Benchmarking

    The expert centres participating in ERKReg continuously follow the performance of their patient management according to key quality indicators of diagnostic and therapeutic interventions.

    ERKReg provides performance statistics for each centre and the entire registry. This allows the centres to compare the quality of their management with that of the overall ERKNet community.

    In this way, we support the excellence and continuous improvement of care in all ERKNet centres throughout Europe.

Have a look at our registry

What data is collected in ERKReg?

General information
Registration identification number Number = pseudonym
Gender
Date of birth Month + year (no day)
Ethnicity For example: Caucasian/ Asian / African, etc
General information regarding the kidney disease
Date of first symptoms Day / Month / Year
Kidney function on that first day Laboratory results: serum creatinine
Other persons within the family with a kidney disease? Yes / No
Clinical diagnosis A number from orphanet will be registered (orphanet is a database of all possible diagnoses) Website orphanet: https://orpha.net/..
Specific information regarding the kidney disease (kidney tissue and/or skin tissue)
Has a kidney biopsy been performed? Yes / No
If yes: Date + diagnosis (conclusion of kidney biopsy report) Immunohistochemical results (this is a specific staining of the biopsy)
Has a skin biopsy been performed? Yes / No
If yes: Date + diagnosis (conclusion of kidney biopsy report) Immunohistochemical results (this is a specific staining of the biopsy)
Specific information regarding the kidney disease (imaging)
Has imaging been performed? Yes / No
If yes: Type of imaging (ultrasound/ MRI/ X-ray)
Specific information regarding the kidney disease (genetics)
If the orphacode (see above) concerns a disease which can be inherited, ERKreg will ask if genetic analysis has been performed.
Has a genetic test been performed? Yes / No
Date of genetic testing Day / Month / Year
Type of genetic test Sanger / gene panel / WES/ WGS (this concerns the technical test which has been performed)
Date of test result Day / Month / Year
Is the test result confirming the clinical diagnosis? Yes / No
If yes: Which gene and which variant has been found? Gene and variant
Specific information regarding the clinical course of the kidney disease (renal replacement therapy)
Has there been a need for renal replacement therapy? Yes / No
If yes: Date of start of renal replacement therapy Day / Month / Year
Type of renal replacement therapy Haemodialysis / peritoneal dialysis / kidney transplantation
If there has been a change in type of renal replacement therapy, it can be registered in ERKreg as well. In that case again the date and type of renal replacement therapy will be registered. Furthermore, the reason of the change will be registered (failure of current therapy or wish of patient/family).
Specific information regarding the clinical follow up (yearly update)
A yearly update about clinical course will take place. Data that will be collected are the following:
Data regarding physical examination:
  • Blood pressure
  • Length / weight
Laboratory measurements:
  • Serum creatinine (kidney function)
  • Urinary protein to creatinine ratio (indication of amount of loss of proteins into urine)
  • Dependant on type of kidney disease results of other laboratory measurements.
At closure of ERKreg participation
When a patient is no longer followed up in the ERKreg registry, a follow-up termination date will be added. Also, the reason of termination of registering data of this particular patient shall be added. Reasons can include: patient withdrawn, patient deceased, or patient cannot be reached due to relocation.
Disease specific information (it will be dependent on type of kidney disease which additional data can be collected)
Data regarding physical examination: For example:
  • Hearing loss?
  • Visual problems?
Laboratory measurements: Extensive laboratory measurements, for example:
  • Immunological tests (e.g. ANA/ANCA/anti-PLA2R)
  • Haemoglobin / leukocytes / platelets
  • Infection parameters
  • Liver function
“Events” For example:
  • Thrombosis
  • Admission to hospital
  • Infections (for instance as a complication when a patient uses immunosuppressive therapy)
  • Death
  • Cancer (which can be a complication of certain treatments of kidney diseases)
Data regarding treatment For example:
  • Immunosuppressive therapy
  • Antihypertensive treatment
  • Anticoagulation
Data regarding the results of treatment This will be data as described above