Is complement a culprit in infection-induced forms of haemolytic uraemic syndrome?

Immunobiology. 2012 Feb;217(2):235-43. doi: 10.1016/j.imbio.2011.07.022. Epub 2011 Jul 26.


Haemolytic uraemic syndrome (HUS) accounts for the most common cause of childhood acute renal failure. Characterized by the classical triad of a microangiopathic haemolytic anaemia, thrombocytopaenia and acute renal failure, HUS occurs as a result of Shiga-toxin producing microbes in 90% of cases. The remaining 10% of cases represent a heterogeneous subgroup in which inherited and acquired forms of complement dysregulation have been described in up to 60%. Emerging evidence suggests that microbes associated with HUS exhibit interaction with the complement system. With the advent of improved genetic diagnosis, it is likely that certain cases of infection-induced HUS may be attributed to underlying defects in complement components. This review summarises the interplay between complement and infection in the pathogenesis of HUS.

Publication types

  • Review

MeSH terms

  • Acute Kidney Injury / immunology
  • Complement Activation / immunology*
  • Complement System Proteins / immunology
  • Hemolytic-Uremic Syndrome / immunology*
  • Hemolytic-Uremic Syndrome / microbiology
  • Hemolytic-Uremic Syndrome / virology
  • Humans
  • Purpura, Thrombotic Thrombocytopenic / immunology
  • Shiga Toxins / metabolism*
  • Thrombocytopenia / immunology


  • Shiga Toxins
  • Complement System Proteins