Life-threatening pregnancy-associated atypical haemolytic uraemic syndrome and its response to eculizumab

Abstract

Pregnancy-associated atypical haemolytic uraemic syndrome (P-aHUS) is a rare, potentially lethal condition that can complicate pregnancy in up to 1 in 25 000 cases. Without prompt diagnosis and initiation of appropriate treatment, this condition can lead to disastrous consequences for both mother and child. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is often difficult to establish the correct diagnosis in a timely manner. Recently, the terminal complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy HUS; however, its use in P-aHUS is limited to isolated case reports. Here, we present a case of fulminant P-aHUS in the postpartum period that was successfully treated with eculizumab resulting in significant recovery of renal function.