Kidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options

Prateek Sanghera, Mythili Ghanta, Fatih Ozay, Venkatesh K. Ariyamuthu, Bekir Tanriover

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)533-538
Number of pages6
JournalAmerican Journal of the Medical Sciences
Volume354
Issue number6
DOIs
StatePublished - Dec 2017

Keywords

  • Alternative complement pathway dysregulation
  • Atypical hemolytic uremic syndrome
  • C3 glomerulopathy
  • Eculizumab

ASJC Scopus subject areas

  • Medicine(all)

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