As part of the innate immune system, the complement system is an important mechanism in our first line of defence, but it can also contribute to the onset of various diseases. In renal diseases, the dysregulation of the complement system is often caused by mutations in-and autoantibodies directed against-members of the complement system, and contributes to disease onset and severity. As the only known positive regulator of the complement system, the role of properdin in complement-mediated diseases is largely unknown. In this review, we provide an overview of the detection of properdin in kidney biopsies and urine, serum or plasma samples from patients with complement-mediated renal diseases, such as immune complex-mediated glomerulonephritis and C3 glomerulopathy. Advances towards a better understanding of the role of properdin in (local) complement activation will provide insight into its potential role and offer opportunities to improve diagnosis and therapeutic interventions.
Keywords: C3 glomerulopathy; complement; glomerulonephritis; kidney; properdin.
© The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.