The CompCure Project and the Registry on C3G and MPGN 

General Information

C3 glomerulopathy (C3G) is a rare kidney disorder arising from a deficiency within the body's complement system. The complement system, an integral part of the body's immune defense against foreign invaders such as viruses and bacteria, functions to eradicate any elements that do not  belong. Normally, the body employs protective proteins to prevent the complement system from targeting its own cells. However, in C3G, this protective mechanism malfunctions, leading to an accumulation of complement components, notably the C3 protein, within the kidney's filtration unit, known as the glomerulus. Over time, this build-up can result in kidney dysfunction, ultimately necessitating dialysis.

Promisingly, novel complement inhibitors are currently in development, promising a breakthrough in the management and outcomes of both IC-MPGN and C3G. Gathering data from individuals afflicted by this rare condition is of utmost importance. This information aids in comprehending the natural progression of the disease, categorizing patients to identify the most effective treatments, and facilitating patient participation in ongoing clinical trials.

Objective

The project's primary objectives are to establish a patient cohort for C3G and MPGN, create a prospective registry for monitoring disease progression, and establish a centralized biobank. 

The data acquisition process for the CompCure project is designed to be comprehensive and accommodating:

  1. Prospective and Retrospective Data Collection: Data collection will be prospective for incident cases, and for prevalent patients with reliable diagnostic data, retrospective information spanning up to three years will be included. This encompasses a wide range of medical, anthropometric, laboratory, and medication details, all systematically recorded in an online database.

  2. Biological Samples Collection: During patients' routine clinical visits, biological samples, including DNA, plasma, serum, and urine, will be collected and securely stored at the Molecular Nephrology Laboratory of the Center for Pediatric and Adolescent Medicine at Heidelberg University.

  3. Specialized Examination for New Patients: New patients and those with missing, incomplete, or outdated diagnostic information will be offered a standardized examination, incorporating immunodiagnostic and genetic assessments. This comprehensive evaluation will be conducted at reference laboratories, with the cost of sending samples covered, ensuring accessibility to all patients without an additional financial burden.

  4. Expert Pathology Review: Digitized scans of biopsy slides, pseudonymized to safeguard privacy, will be transmitted to the expert pathology center specializing in C3G. Here, a reference pathology review will be conducted, enhancing the diagnostic process.

  5. Direct Communication of Laboratory Results: All laboratory results will be communicated directly to the attending physician, who will be responsible for sharing this information with the patient while ensuring that the data is pseudonymized before entering it into the online registry.

  6. Biobank Samples and Future Reanalysis: Samples stored in the biobank will be maintained and may be reanalyzed in the future, responding to advancements and discoveries in the fields of immunological and genetic diagnostics. This forward-thinking approach ensures that the data remains relevant and useful over time.

Inclusion Criteria

The registry welcomes both pediatric and adult patients of any age who have been diagnosed with C3G or MPGN.  Eligibility for enrollment is contingent on the availability of well-documented laboratory and histopathological data at the time of diagnosis.

Patients with a secondary C3G/MPGN are excluded from the study.

 

General information about the CompCure Registry on C3G/MPGN

The CompCure Registry on C3G and MPGN is a subregistry of the ERKNet registry (ERKReg). This registry can be completed when a patient is included in the baseline data sheet. In addition, in collaboration with ESPN centers, patients from non-ERKReg member sites will also be included.

The CompCure project encompasses the creation of a registry and biobank. As part of this initiative, C3G/MPGN patients who wish to participate in the Cohort study, must provide consent for the use of data in ERN registries and databases (ERKReg). Patients are also required to grant their approval for the collection of blood and urine samples, as stipulated in the CompCure informed consent.

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

 

Ethics approval

If you are an ERKNet member, your center will already have ethics approval for ERKReg and subregistrations including CompCure. You must, however, obtain additional consent to collect biological samples for the biobank.

If you are not a member of ERKNet: be sure to obtain ethics approval before entering patients.

 

Compilation of the CompCure Registry on C3G and MPGN

Basic data collection

General background information includes the date of informed consent (1) consent to share data for clinical care, (2) consent to include coded data in one or more ERN databases, and (3) consent to be contacted for research projects.

Key data are sex, date of birth (mm/yy), ethnicity (pop-up menu), date of first symptoms, date of first presentation to the center, and clinical symptoms at the time of disease onset.

In addition, the primary renal diagnosis, histopathologic diagnosis, autoimmunologic diagnostic and genetic test results are indicated.

When the basic data are saved, the unique ID number is provided. The patient cannot be recognized in any way. Only the attending physician at the home center is able to append the patient's name with the ID.  

Annual visits, at change of therapy and three months later.

Annual baseline questions include anthropometric and renal function data, laboratory blood results, comorbidities, and complications. 

 

Medications

Look for medication in the list --> if you cannot find your medication, please contact us (giulia.bassanese@med.uni-heidelberg.de).

Enter the start date and end date (if in progress leave blank).

A list of past medications (marked in red) and current medications (marked in green) will be displayed. 

Don't forget to fill in data on induction treatment and medications administered between visits.

Don't forget to include the reasons for discontinuation of treatment.

 

Data dictionary 

The data dictionary of ERKReg, including the collected parameters of the CompCure subregistry, can be found here. 

 

Hospitalizations

Users  are encouraged to record any hospitalizations they experience, along with the specific reasons for their admission to the hospital. This information is valuable for maintaining comprehensive medical records and understanding the patient's health history.

 

For more information please visit the CompCure Website: www.compcure.org or contact:  giulia.bassanese@med.uni-heidelberg.de, msn@compcure.org