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

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3 Citations (Scopus)

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)
JournalAmerican Journal of the Medical Sciences
DOIs
StateAccepted/In press - Jan 1 2017

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Alternative Complement Pathway
Kidney Diseases
Complement Factor H
Complement Inactivating Agents
Complement Factor I
CD46 Antigens
Membranoproliferative Glomerulonephritis
Therapeutics
Glomerulonephritis
Kidney Transplantation
Autoimmune Diseases
Chronic Kidney Failure
Allografts
Proteins
Quality of Life
Transplants
Kidney
Pregnancy
Mutation
Infection

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)

Cite this

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title = "Kidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options",
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.",
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author = "Prateek Sanghera and Mythili Ghanta and Fatih Ozay and Ariyamuthu, {Venkatesh K.} and Bekir Tanriover",
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AU - Ghanta, Mythili

AU - Ozay, Fatih

AU - Ariyamuthu, Venkatesh K.

AU - Tanriover, Bekir

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