Thrombotic microangiopathies (TMAs) and complement-mediated kidney diseases are rare but serious conditions. They are associated with damage to the small blood vessels and a dysregulation of the complement system (an important part of the immune system). These conditions include atypical haemolytic uraemic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP), C3 glomerulopathy and immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). They can manifest both as acute, potentially life-threatening events and as chronic, progressive kidney diseases.
In recent years, there has been significant progress in the development of new therapies. In particular, the development of targeted complement inhibitors and innovative enzyme replacement therapies (for example with recombinant ADAMTS13 for the treatment of congenital TTP) has substantially improved patient care.
As all of these diseases are very rare, only limited data on disease courses, long-term outcomes and care structures are available. This makes it difficult to develop evidence-based treatment strategies and to investigate new therapeutic approaches. The Registry for Complement-Mediated Kidney Diseases and Thrombotic Microangiopathies (CORE registry) aims to help close this knowledge gap.
The CORE registry records patients with TMAs and complement-mediated kidney diseases throughout Germany. Both children and adults can be enrolled.
Through the systematic collection of clinical, laboratory, histopathological and genetic data, disease courses are to be comprehensively characterised and a better understanding of the underlying disease gained.
Due to the national scope of the registry, a larger number of patients can be enrolled despite the rarity of these diseases, in order to obtain scientifically robust data.
The aim of the registry is to gain new scientific insights into disease mechanisms and to better assess the significance of new therapeutic approaches. In the long term, the data collected in the CORE registry should help to deepen the understanding of these rare diseases, to individualise treatment decisions more strongly and to sustainably improve the prognosis and care of affected patients.
Participation in the CORE registry is open to all patients with TMAs and complement-mediated kidney diseases.
Find your nearest CORE centre with expertise in the treatment of TMA or complement-mediated kidney diseases here. Participation in the CORE registry is entirely voluntary, and the study does not involve any changes to your therapy. It is solely about recording your medical findings and the course of your disease, retrospectively (i.e. what happened before the start of the study) and prospectively (i.e. what happens from the time you join the study). No specific study appointments are required either; the data are collected during your regular check-ups at your kidney centre. The entries are made by your treating physicians on the basis of your consent. Optionally, you can also provide blood, urine and stool samples for research purposes during routine examinations. This biomaterial is stored in local biobanks and can be used for further research projects within the CORE registry.
If your treating nephrology centre does not yet participate in the CORE registry, you can either contact us directly or draw your treating physicians' attention to the registry.