Eculizumab as an adjunctive rescue treatment for life-threatening childhood onset of systemic lupus erythematosus
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.
Authors
- Teresa Giani (ORCID: https://orcid.org/0000-0001-9292-7601)
- Gabriele Simonini (ORCID: https://orcid.org/0000-0002-5919-4861)
- Ilaria Pagnini (ORCID: https://orcid.org/0000-0002-4508-2017)
- Ilaria Maccora (ORCID: https://orcid.org/0000-0001-6418-3254)
- Maria Vincenza Mastrolia (ORCID: https://orcid.org/0000-0002-9784-3543)
- Edoardo Marrani (ORCID: https://orcid.org/0000-0002-0346-3268)
- Antonio Tiezzi
- Linda Zollo
Institutions
- Meyer Children's Hospital (IL)
- Meyer Children's Hospital (IT)
- Istituti di Ricovero e Cura a Carattere Scientifico (IT)
- University of Florence (IT)
Publication Details
- Journal
- Lupus
- Published
- 2025-11-24
- DOI
- https://doi.org/10.1177/09612033251399945
- Primary Topic
- Complement system in diseases
- Type
- article
- Field-Weighted Citation Impact
- 0.00