Kidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options. Review uri icon

Overview

abstract

  • Atypical hemolytic uremic syndrome and C3 glomerulopathy (dense deposit disease and C3 glomerulonephritis) are characterized as inappropriate activation of the alternative complement pathway. Genetic mutations affecting the alternative complement pathway regulating proteins (complement factor H, I, membrane cofactor protein and complement factor H-related proteins) and triggers (such as infection, surgery, pregnancy and autoimmune disease flares) result in the clinical manifestation of these diseases. A decade ago, prognosis of these disease states was quite poor, with most patients developing end-stage renal disease. Furthermore, renal transplantation in these conditions was associated with poor outcomes due to graft loss to recurrent disease. Recent advances in targeted complement inhibitor therapy resulted in significant improvement in disease remission, renal recovery, health-related quality of life and allograft survival.

publication date

  • March 16, 2017

Research

keywords

  • Complement Pathway, Alternative
  • Kidney Diseases

Identity

PubMed Central ID

  • PMC5720382

Scopus Document Identifier

  • 85033448597

Digital Object Identifier (DOI)

  • 10.1016/j.amjms.2017.03.024

PubMed ID

  • 29208248

Additional Document Info

volume

  • 354

issue

  • 6