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The terminal complement inhibitor eculizumab has improved the outlook for patients with atypical hemolytic uremic syndrome (aHUS), especially with regard to development of advanced renal disease. Most patients are treated indefinitely to reduce risk for relapse, but such treatment is associated with risk for meningitis, increased costs, and need for infusions every 2 weeks. Investigators designed a multicenter uncontrolled prospective study to assess the incidence of aHUS relapse during 2 years of follow-up after eculizumab discontinuation.
The study included 55 patients (19 children, 38 adults). Key findings include:
The mean duration of eculizumab treatment was 16.5 months. Prior to discontinuation, 9 patients (16%) had more than one aHUS e…