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Diseases of complement dysregulation-an overview.

Edwin K S Wong1,2, David Kavanagh3,4

  • 1The National Renal Complement Therapeutics Centre, aHUS Service, Building 26, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.

Seminars in Immunopathology
|January 13, 2018
PubMed
Summary

Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are complement disorders. New therapies targeting complement dysregulation show promise for these conditions.

Keywords:
C3G, aHUSComplementPNH

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Area of Science:

  • Nephrology
  • Hematology
  • Immunology

Background:

  • Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are key disorders of complement dysregulation.
  • While complement overactivation is a common feature, the specific pathway affected (alternative or terminal) and the resulting clinical phenotype differ among these diseases.

Purpose of the Study:

  • To review the distinct mechanisms of complement dysregulation in aHUS, C3G, and PNH.
  • To discuss the therapeutic landscape, including the efficacy of eculizumab and the potential of next-generation complement therapeutics.

Main Methods:

  • Review of existing literature on aHUS, C3G, and PNH.
  • Analysis of the underlying genetic and autoimmune mechanisms driving complement dysregulation.
  • Evaluation of current and emerging complement-targeted therapies.

Main Results:

  • aHUS involves cell surface alternative pathway dysregulation often due to inherited mutations.
  • C3G is characterized by fluid phase alternative pathway dysregulation, frequently linked to autoimmune factors.
  • PNH results from terminal pathway dysregulation driven by somatic mutations, effectively treated by eculizumab, which has shown less success in C3G.

Conclusions:

  • Understanding the specific complement dysregulation in aHUS, C3G, and PNH is crucial for targeted therapy.
  • Next-generation complement therapeutics are anticipated to build upon the success seen with eculizumab, particularly for aHUS and PNH, and potentially offer new avenues for C3G treatment.