In recent years, the complement system, which plays a crucial role in the innate immunity, fostered renewed scientific interest. This process has brought a rare kidney disorder, named C3 glomerulopathy, in the center of a real revolution, highlighting the complex interactions between genotype, triggers and kidney microenvironment, which together contribute to the final phenotype of the disease. At the same time, experimental studies and clinical trials testing new complement inhibitors have multiplied enormously. It’s a very promising time for patients affected by C3 glomerulopathy, which didn’t have so far effective therapies in preventing the progression to the end-stage kidney disease.
Keywords: Membranoproliferative glomerulonephritis, C3 glomerulopathy, complement system, alternative pathway, kidney biopsy