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Atypical hemolytic uremic syndrome.

David Kavanagh1, Tim H Goodship, Anna Richards

  • 1The Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.

Seminars in Nephrology
|October 29, 2013
PubMed
Summary

Atypical hemolytic uremic syndrome (HUS) involves complement overactivation due to genetic defects. Triggers can unmask these deficiencies, impacting prognosis, but eculizumab offers revolutionary treatment for this rare disease.

Keywords:
Complementeculizumabfactor Hfactor Ihemolytic uremic syndromemembrane cofactor proteinthrombomodulintransplantation

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Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Hemolytic uremic syndrome (HUS) is defined by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.
  • Atypical HUS (aHUS) is characterized by dysregulation of the complement system, a critical part of the immune system.
  • Genetic predispositions, including inherited complement gene defects and autoantibodies against complement proteins, underlie aHUS.

Purpose of the Study:

  • To summarize the pathophysiology of atypical hemolytic uremic syndrome (aHUS).
  • To discuss the role of complement system dysregulation in aHUS.
  • To highlight the prognostic implications of genetic defects and the therapeutic potential of complement inhibition.

Main Methods:

  • Literature review of studies on atypical hemolytic uremic syndrome.
  • Analysis of genetic defects and complement system pathways involved in aHUS.
  • Evaluation of clinical trial data for eculizumab in aHUS treatment.

Main Results:

  • Incomplete penetrance of genetic mutations suggests a requirement for precipitating events to trigger aHUS.
  • Underlying genetic defects are crucial for predicting patient outcomes in both native kidneys and post-transplant settings.
  • Eculizumab has demonstrated significant success in clinical trials for treating aHUS.

Conclusions:

  • Atypical HUS pathogenesis is linked to complement overactivation, often influenced by genetic factors and environmental triggers.
  • Genetic profiling is essential for understanding aHUS prognosis and guiding management strategies.
  • The complement inhibitor eculizumab represents a paradigm shift in managing atypical HUS, offering improved patient outcomes.