Atypical haemolytic
uremic syndrome (aHUS)

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare, yet life-threatening condition linked to thrombotic microangiopathy (TMA). Early recognition and accurate diagnosis are critical to improving patient outcomes.1

Atypical haemolyticuraemic syndrome (aHUS) is a form of thrombotic microangiopathy (TMA) caused by overactivation of the terminal complement system.1 The overactivation of the complement system triggers a destructive cycle of endothelial damage and dysfunction which leads to thrombosis, destruction of erythrocytes and organ damage and dysfunction. aHUS is a life-threatening condition and can lead to stroke, heart attack, kidney failure and premature death.1-2,4-6

ahus-overview
aHUS can be precipitated by factors or conditions that trigger complement activation and/or a patient’s genetic predisposition to complement dysregulation.1,11,12

In some patients, there may be no identified genetic variant; 30–50% of aHUS patients do not have an identified disease-causing genetic driver. Genetic testing is not required for a diagnosis of aHUS.2,7-10

Potential triggers include:1–5

Pregnancy / postpartum
Malignant hypertension
Organ/bone marrow transplantation
Autoimmune diseases
Glomerulonephritis
Malignancy
Infections
Certain medications
Surgery/trauma

aHUS can lead to:1–2

Nausea/vomiting
Pain and blurred vision
Elevated creatine level
Kidney failure
Seizures
Hypertension
Dyspnoea
aHUS is a medical emergency – diagnosis and initiation of appropriate treatment for aHUS should not be delayed while awaiting identification of the responsible trigger or aetiology.2

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References:

  • Laurence J, et al. Clin Adv Hematol Oncol. 2016;14:2–15.
  • Azoulay E, et al. Chest. 2017;152:424–434.
  • Nester CM, Thomas CP. Hematology Am Soc Hematol Educ Program. 2012;2012:617–625.
  • Goodship THJ, et al. Kidney Int. 2017;91:539–551.
  • Bommer M, et al. Dtsch Arztebl Int. 2018;115:327–334.
  • Hofer J, et al. Front Pediatr. 2014;2:97.
  • Afshar-Kharghan V. Hematology Am Soc Hematol Educ Program. 2016;2016:217–225.
  • Noris M, et al. Clin J Am Soc. Nephrol. 2010;5:1844–1859.
  • Loirat C, et al. Pediatr. Nephrol. 2016;31(1):15-39
  • Noris M, et al. Genetic atypical hemolytic-uremic syndrome. Available at:https://www.ncbi.nlm.nih.gov/books/NBK1367/
  • Chatzikonstantinou T, et al. Front Med (Lausanne). 2020;7:212.
  • Vande Walle J, et al. J Nephrol. 2017;30(1):127–134.