aHUS Pregnancy Eculizumab Immediate Treatment — ESENeph MCQ

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HardPregnancy & RenalaHUS Pregnancy Eculizumab Immediate TreatmentESENeph

A 34-year-old woman at 26 weeks' gestation develops severe abdominal pain and dark urine. She has a known complement factor H mutation. Bloods: platelets 55 x 10^9/L, LDH 2200 U/L, haptoglobin <0.1 g/L, creatinine 380 umol/L. Schistocytes present on film. ADAMTS13 activity is 72%. She had a similar episode postpartum with her first child. What treatment should be initiated immediately?

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Correct answer: A — Eculizumab (anti-C5 monoclonal antibody)

This presentation — TMA with normal ADAMTS13, known complement factor H mutation, and previous postpartum episode — is atypical HUS (complement-mediated TMA). Eculizumab (anti-C5 monoclonal antibody) is the treatment of choice and should be started immediately. It blocks terminal complement activation, halting endothelial injury. Unlike HELLP/pre-eclampsia (where delivery is treatment), aHUS requires specific complement blockade. Plasma exchange may be used as a bridge but is not definitive for aHUS. Platelet transfusion can worsen TMA. Delivery alone will not resolve complement-mediated disease. Eculizumab use in pregnancy is supported by case series and expert consensus.

Reference: https://guidelines.ukkidney.org