Atypical aHUS: State of the Art

Mol Immunol. 2015 Sep;67(1):31-42. doi: 10.1016/j.molimm.2015.03.246. Epub 2015 Apr 3.

Abstract

Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.

Publication types

  • Review

MeSH terms

  • Adult
  • Atypical Hemolytic Uremic Syndrome / etiology
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Atypical Hemolytic Uremic Syndrome / immunology
  • Atypical Hemolytic Uremic Syndrome / pathology*
  • Autoantibodies / biosynthesis
  • Child
  • Communicable Diseases / complications
  • Communicable Diseases / genetics
  • Communicable Diseases / immunology
  • Communicable Diseases / pathology*
  • Complement Activation
  • Complement C3b / genetics
  • Complement C3b / immunology
  • Complement C3b Inactivator Proteins / genetics
  • Complement C3b Inactivator Proteins / immunology
  • Complement Factor H / genetics
  • Complement Factor H / immunology
  • Gene Expression Regulation / immunology*
  • Genetic Predisposition to Disease
  • Humans
  • Kidney / immunology
  • Kidney / pathology*
  • Kidney Neoplasms / complications
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / immunology
  • Kidney Neoplasms / pathology*
  • Kidney Transplantation

Substances

  • Autoantibodies
  • Complement C3b Inactivator Proteins
  • complement factor H, human
  • Complement C3b
  • Complement Factor H