ERKNet/ESPN pediatric SLE nephritis registry

General information

Lupus (SLE) Nephritis

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease of unclear etiology, which can be directly mediated by autoantibodies or indirectly affect all organ systems through immune complex deposition. Kidney involvement occurs in 60- 80% of paediatric SLE patients. Children presenting with lupus nephritis (LN) are treated with various immunosuppressive protocols including long-term steroid treatment. Pediatric patients are mostly treated off-label since Data on renal outcome and treatment toxicity are scare. Development specific requirements of paediatric patients are not taken into account. Not only growth and development, but also psychological factors such as puberty and family structures differentiate this group of patients from adults.

The objective of ESPN - ERKNet LN registry is to collect data on long term renal outcome and its determining factors in childhood onset LN (including transition) and detect disease and treatment associated comorbidities in order to allow benchmarking, develop research hypothesis, improve treatment and outcome. The register will be mainly prospective, retrospective data since 2015 will also be included.

The LN Registry is an affiliated Registry of ERKReg. This registry can be filled in when a patient is included in the basic data sheet. Additionally, patients from Non-ERKReg member sites will be included in collaboration with ESPN centres.


General information on the Pediatric SLE Nephritis Registry

The LN Registry is a subregistry of the ERKNet registry (ERKReg). One of the objectives of ERKNet is to improve knowledge on rare kidney diseases.

The LN Subregistry is started to gather information on the symptoms at diagnosis, course of the disease, treatment modalities and long-term outcome of the patients. Special emphasis is placed on disease and treatment associated comorbidities and complications in order to to help optimizing and monitoring the quality of patient care.

Patients must give a consent for data use in ERN registries and databases. Non-ERN members will also have to give consent for data use for the LN registry.

Every patient enrolled will have a unique ID code (number of center ID number  as 001-001). A local document with name, surname, date of birth, date of informed consent and ID, will be stored on the hospital server in a secured location.


Ethical approval

  • If you are an ERKNet member: your center will already have ethical consent for the ERKReg. Please make sure to check with your ethics committee if additional must be obtained for the sub-registry.
  • If you are not an ERKNet member: please make sure to obtain ethical approval before entering patients.
  • All centres: Attached you will find a template for ethics request and the informed consent form. The consent form includes consent for the ERKReg and on the second page specific consent for the subregistries.

Filling in your patients

Basic data collection

The general basic information includes the date of informed consent (1) the consent to data being shared for clinical care, (2) the consent to coded data being included in one or more ERN databases and (3) the consent to being contacted about research projects.

Inclusion criteria are a diagnosis of LN under the age of 18 years and biopsy proven LN. This means adult patients can be included as long as the diagnosis was established under 18 years.

The basic data are the sex, the date of birth (mm/yy), the ethnicity ( popup menu), the date of first symptoms and the date of the first presentation to center, parents height, pubertal status at disease onset (not all questions are mandatory).

Primary renal diagnosis: The diagnosis is prefilled and can’t be changed (childhood onset systemic lupus erythematosus).

When was the diagnosis considered confirmed:  please enter date of biopsy. If diagnosis was established by clinical score (i.e. ACR/EULAR, SLICC) enter the date of clinical diagnosis.

Clinical examination at time of diagnosis: symptoms are based on SLEDAI Score (https://rheumatologie.at/pdf/sledai.pdf)

Histopathological diagnostics: The diagnosis is prefilled and can’t be changed. Please enter both Classification if known.

When basic data are saved, the unique ID number will be provided and a visit can be filled in (annual visits).  “Add visit”

 

Annual visits

Annual basic questions encompasses data on anthropometrics and renal function, blood results and complications. Annual ophtalmological and cardiological findings will be asked. symptoms are based on SLEDAI Score (https://rheumatologie.at/pdf/sledai.pdf).

Laboratory results: if you don’t find the unit used at your center please don’t hesitate to contact us!


Medications

  1. Search the medication in the list --> if you don’t find your medication please don’t hesitate to contact us (suhlrie.adriana[at]mh-hannover.de)
  2. Enter start date and stop date (if ongoing leave empty)

You will have a list of past medication (marked red) and ongoing medication (green). Immunosuppressants and steroids will be asked in accurate dosage, others as selection yes/no.

Please don’t forget to fill in induction treatment and medication in between the visits.

Please don’t forget to fill in reasons for termination


Thank you a lot for your help and cooperation!



European Reference Networks This website is part of the project / joint action "777304 / ERK-REG" which has received funding from the European Union’s Health Programme (2014-2020)