Atypical Haemolytic Uraemic Syndrome (aHUS) is an ultra-rare, life-threatening disease that severely damages vital organs, including the kidneys, heart and brain.1,2,3aHUS is caused by chronic, uncontrolled activation of the complement system, (part of the body’s immune system) leading to the formation of micro blood clots in small blood vessels throughout the body.
The accumulation of micro blood clots and the associated damage and swelling within small blood vessels is known as thrombotic microangiopathy.
- aHUS induced TMA can occur in any part of the body, causing vital organs to fail.5 TMA can also lead to stroke, heart attack, kidney failure and death.
- aHUS can occur at any age; with nearly half all people diagnosed aged 18 and older. This disease is as life-threatening in adults as it is in children.
- Between 33 and 40 per cent of people diagnosed with aHUS will die or progress to ESRD following their first clinical manifestation.
- 10 to 15 per cent of all patients with aHUS die within the first year of diagnosis (mortality in patients within some groups is 19 per cent) and 32 percent die within four years.
- Within one year of diagnosis 64 per cent of patients with aHUS will die, require dialysis or develop permanent kidney damage despite plasma exchange.8,9
- In 70 per cent of cases, aHUS is associated with a genetic or acquired abnormality of a part of the immune system known as the ‘complement system’, (30 per cent remain unexplained) which can lead to severe inflammation of the blood vessels and blood clotting that damages the kidneys, causing them to fail.1,4,7,10
- Australian aHUS medical registries report a 17 per cent annual mortality rate.