Childhood-onset systemic lupus erythematosus (cSLE) often presents with aggressive manifestations that may not respond to standard immunosuppressive therapy. We describe two paediatric cSLE cases with severe life-threatening complications—autoimmune hemolytic anemia in one patient and macrophage activation syndrome with acute respiratory distress syndrome in the other—successfully managed with Eculizumab as adjunctive treatment. Both patients demonstrated rapid stabilization after Eculizumab initiation and showed favorable outcomes at their latest follow-up. These cases highlight the potential role of complement inhibition in select cSLE complications and suggest that Eculizumab may serve as a rescue therapy beyond its established use in thrombotic microangiopathy. Further studies are needed to better define its optimal use and target patient population.
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Tiezzi et al. (2025) studied this question.
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