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Haemolytic uraemic syndrome (HUS) is characterized by microangiopathic haemolytic anaemia with acute kidney injury. It is currently classified into two main categories: Shiga-toxin producing E. coli-haemolytic uraemic syndrome (STEC-HUS) and atypical haemolytic uraemic syndrome (aHUS). Endothelial cell damage is the common pathway in HUS to developing thrombotic microangiopathy. Atypical HUS includes primary, secondary and aHUS due to metabolic diseases. In the majority of aHUS cases, hyperactivity of the alternative complement pathway plays a central role. Therefore, treatment is based on complement inhibitors like eculizumab, a drug that has revolutionized the natural history of the disease. Relapses are frequent after kidney transplant and thus confer a poor prognosis.