Atypical HUS — ESENeph MCQ

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HardInherited and Rare Renal DiseaseAtypical HUSESENeph

A patient with complement-mediated atypical HUS has a pathogenic CFH variant and has not received a complement inhibitor. Which long-acting NHS option is recommended by NICE?

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Correct answer: B — Start terminal-complement inhibition with ravulizumab

The best answer is “Start terminal-complement inhibition with ravulizumab”. NICE recommends ravulizumab for untreated aHUS and for people responding after at least 3 months of eculizumab. Its prolonged terminal-complement inhibition reduces infusion frequency. “Caplacizumab” is less appropriate because caplacizumab targets von Willebrand factor in acquired TTP “Rituximab as isolated complement blockade” is less appropriate because rituximab does not inhibit terminal complement “Plasma exchange as definitive lifelong prevention for every CFH variant” is less appropriate because terminal complement inhibition is the disease-specific treatment “Tolvaptan” is less appropriate because tolvaptan slows progression in selected ADPKD

Reference: NICE TA710: ravulizumab for atypical haemolytic uraemic syndrome: https://www.nice.org.uk/guidance/ta710/chapter/1-Recommendations