Atypical hemolytic uremic syndrome (aHUS), also known as complement-mediated hemolytic uremic syndrome, is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. In most cases it can be effectively controlled by interruption of the complement cascade. Particular monoclonal antibodies, discussed later in the article, have proven efficacy in many cases.
AHUS is usually caused by chronic, uncontrolled activation of the complement system,[1][2] a branch of the body's immune system that destroys and removes foreign particles.[3] The disease affects both children and adults and is characterized by systemic thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to stroke, heart attack, kidney failure, and death.[1][4][5] The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane cofactor protein),[6][5][7] or is occasionally due to acquired neutralizing autoantibody inhibitors of these complement system components, for example anti–factor H antibodies.[8]: 1933 Prior to availability of eculizumab (Soliris) and ravulizumab (Ultomiris), an estimated 33–40% of patients died or developed end-stage renal disease (ESRD) (despite the use of supportive care, e.g. plasmapheresis) with the first clinical bout of aHUS. Including subsequent relapses, a total of approximately two-thirds (65%) of patients died, required dialysis, or had permanent renal damage within the first year after diagnosis despite plasma exchange or plasma infusion (PE/PI).[7]
^ abCite error: The named reference Loirat2008 was invoked but never defined (see the help page).
^Nester, Carla M.; Thomas, Christie P. (8 December 2012). "Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated?". ASH Education Program Book. 2012 (1): 617–625. doi:10.1182/asheducation.v2012.1.617.3798924. ISSN 1520-4391. PMID 23233643.
^Cite error: The named reference Walport2001 was invoked but never defined (see the help page).
^Cite error: The named reference Hosler2003 was invoked but never defined (see the help page).
^ abCite error: The named reference Noris2010 was invoked but never defined (see the help page).
^Tzoumas, Nikolaos; Hallam, Dean; Harris, Claire L.; Lako, Majlinda; Kavanagh, David; Steel, David H.W. (November 2020). "Revisiting the role of factor H in age-related macular degeneration: Insights from complement-mediated renal disease and rare genetic variants". Survey of Ophthalmology. 66 (2): 378–401. doi:10.1016/j.survophthal.2020.10.008. ISSN 0039-6257. PMID 33157112. S2CID 226274874.
^ abCite error: The named reference Caprioli2006 was invoked but never defined (see the help page).
^Cite error: The named reference Hoffman_2012_6 was invoked but never defined (see the help page).
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