Eculizumab in Hypertensive Emergency-associated Hemolytic Uremic Syndrome (HYPERSHU)

Inclusion Criteria: ≥ 18years Hospitalization for HE-aHUS within prior 10 days: Presume acute renal failure (renal replacement therapy or serum creatinine ≥ 354µM) Mechanical hemolysis including: anemia, thrombopenia, and: low haptoglobin (<LNL), or elevated LDH (>1,5UNL), or presence of schistocytes Severe hypertension with systolic blood pressure >180mmHg or diastolic blood pressure>110mmHg Target organ damage, including neurological involvement (notably hypertensive encephalopathy, headache, confusion, nausea, posterior reversible encephalopathy syndrome), or cardiovascular involvement (notably acute left ventricular failure, acute pulmonary edema, acute cardiac ischemia, chest pain, dyspnea, palpitations), or ophtalmological involvement (notably ischemic retinopathy or blurred vision) Effective contraception during the study and for at least 5 months after the last dose of treatment with eculizumab Subject affiliated to a social security regimen Subject having signed written informed consent. Exclusion Criteria: Atrophic kidneys with maximum length<8cm on recent (<1 month) renal ultrasound, CT scan, or renal MRI High clinical suspicion of Complement-mediated aHUS (including familial history of aHUS) High clinical suspicion of typical HUS (including Shiga Toxin-producing E. Coli infection) or Thrombotic thrombocytopenic purpura High clinical suspicion of secondary HUS related to autoimmune disease (including lupus, scleroderma, antiphospholipid syndrome, ANCA vasculitis), or C3 glomerulopathy. High clinical suspicion of recent hemorrhagic or ischemic stroke. ADAMTS 13<10%, HIV or HCV infection, positivity of 2 markers among: anticardiolipin IgG/antiBeta2 GP1 IgG/lupus anticoagulant, positivity of ANCA (ELISA PR3 or MPO) Active infection Subjects with unresolved Neisseria meningitidis infection Subjects refusing Neisseria meningitidis vaccination or refusing antibioprophylaxis with oracillin (In case of penicillin allergy, antibioprophylaxis with macrolide couldbeproposed according to ANSM recommendations (azithromycin or roxithromycin)). Contra-indication to eculizumab or renin angiotensin system blockers Solid organ or haematopoietic transplant History (<1year) of active cancer or exposition to drugs associated with aHUS (< 3 months) Severe cognitive or psychiatric disorders, patients unable to give an informed consent. PCR SARS-CoV2 positive Pregnant or breastfeeding woman or ineffective contraception Persons deprived of their liberty by judicial or administrative decision, Persons under legal protection (guardianship, curatorship) Participation in another interventional study involving human participants or being in the exclusion period at the end of a previous study involving human participants.