Pregnancy-associated hemolytic uremic syndrome

Abstract EN

Pregnancy-associated hemolytic uremic syndrome (P-aHUS) is not an uncommon condition.

It is considered a medical emergency that is associated with a high risk of mortality and serious morbidity.

End-stage renal disease as a consequence of P-aHUS occurs in >50% of the patients if left untreated; the majority of identified cases (79%) are during the postpartum period.

Its mechanism of action is related mainly to the disturbance in the activation of the complement alternative pathway, leading to damage of the microvascular endothelium.

The clinical picture of P-aHUS mimics several conditions occurring during post-partum thrombotic microangiopathy, for example, severe pre-eclampsia, hemolysis, elevated liver enzymes, and low platelet count, thrombotic-thrombocytopenic purpura, and acute fatty liver of pregnancy.

Genetic analysis of known genetic mutations together with the analysis of anti-CFH antibodies might confirm the diagnosis of aHUS in the post-partum period.

The absence of causative genetic mutations does not always exclude a diagnosis of aHUS, since 40% of patients show no known genetic abnormalities.

The mainstay of management is supportive care and immediate initiation of plasmapheresis.

Eculizumab has been proved to be both safe and effective in inducing and maintaining remission in P-aHUS and it is recommended to be started as soon as the diagnosis is established.