Author:

Keywords:

Science & Technology, Life Sciences & Biomedicine, Medicine, General & Internal, General & Internal Medicine, C3 glomerulonephritis, C3 glomerulopathy, center of expertise, dense deposit disease, membranoproliferative glomerulonephritis, DENSE DEPOSIT DISEASE, MONOCLONAL GAMMOPATHY, ALTERNATIVE PATHWAY, MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS, NEPHRITIC FACTORS, ECULIZUMAB, DYSREGULATION, VARIANTS, OUTCOMES, Complement Activation, Complement C3, Complement Pathway, Alternative, Glomerulonephritis, Humans, Kidney, Kidney Glomerulus

Abstract:

C3 glomerulopathy is a rare renal disease that has been distinguished as a renal disease for about 10 years. It is caused by an excessive activation of the alternative complement pathway in the circulation, which leads to deposition of complement factor C3 in glomeruli. It is diagnosed based on clinical presentation, histological patterns in a kidney biopsy and tests of the complement pathways. It can closely resemble immune complexmediated glomerulonephritis and postinfectious glomerulonephritis. Renal failure develops in up to half of all patients within 10 years after presentation. A curative treatment is not available. Treatment relies on renoprotective measures, occasional immunosuppressive medication and experimental novel complement inhibitors. Because the disease is rare, its care and cure are concentrated in centers of expertise. Here we provide an overview of the state-ofthe-art diagnosis and treatment of C3 glomerulopathy in a center of expertise in the Netherlands.