Kidney Disease MPGN II/DDD/C3G
Membranoproliferative Glomerulonephritis (MPGN) is a rare kidney disorder which most frequently affects young patients. This disease which is currently also termed C3 glomerulopathy represents a spectrum of related disorders. The major diagnosis is based on the immunohistological evaluation of a kidney biopsy.The involvement of complement and autoimmune parameters such as the C3 nephritic factor, autoantibodies to Factor B, Factor H and to the C3 convertase have frequently been reported. In addition, genetic mutations including the genes Factor H, C3 and the CFHR genes, i.e. CFHR2, CFHR5 and CFHR3 are reported in patients with MPGN /C3G. The Department of Infection Biology is interested in understanding the exact role of complement in the pathomechanisms of this severe kidney disorder. Our central aim is to understand the scenarios that result in the formation of autoimmune forms. In addition, we aim at defining how the various genetic alterations result in pathology and the heterogeneity of this disease. To allow a better understanding of the heterogeneity and pathophysiology we have established a registry that currently includes more than 250 patients suffering from the initial diagnosis MPGN I, or MPGNII. This allows us to define subtypes of the heterogeneous disease. Individual auto antibodies and mutant proteins are characterized to show how they affect and disrupt immune homeostasis. Using this approach and in close collaboration with clinical and medical doctors, new forms of diagnosis and therapy are being developed with regard to these severe kidney diseases.