ArticlePediatric nephrology (Berlin, Germany)2015
Dramatic effects of eculizumab in a child with diffuse proliferative lupus nephritis resistant to conventional therapy.
Article in Pediatric nephrology (Berlin, Germany), 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 3 of them syntheses that pooled it.
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Who cites it
36 citing papers in PubMed, 3 syntheses or guidelines pooled it, 75 citations in OpenAlex.
- Complement-amplified thrombotic microangiopathy in systemic lupus erythematosus and lupus nephritis: a systematic review of 78 reported patients.Frontiers in immunology · 2026Pooled it
- A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy.BMC nephrology · 2020Pooled it
- Use of eculizumab in a systemic lupus erythemathosus patient presenting thrombotic microangiopathy and heterozygous deletion in CFHR1-CFHR3. A case report and systematic review.Clinical rheumatology · 2017Pooled it
- Complement-mediated thrombotic microangiopathy associated with lupus nephritis.Blood advances · 2018Trial
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Case Report: Sequential eculizumab and belimumab in a paediatric patient with lupus nephritis and immune complex-mediated renal microangiopathy.Frontiers in immunology · 2026Article
- Vascular injury in glomerulopathies: the role of the endothelium.Frontiers in nephrology · 2024Review
- Immune podocytes in the immune microenvironment of lupus nephritis (Review).Molecular medicine reports · 2023Review
- Low-molecular weight inhibitors of the alternative complement pathway.Immunological reviews · 2023Review
- Article
- Retinal Drusen Are More Common and Larger in Systemic Lupus Erythematosus With Renal Impairment.Kidney international reports · 2022Article
- Review
- COVID-19 and acute kidney injury in pediatric subjects: is there a place for eculizumab treatment?Journal of nephrology · 2020Article
- The Complement System in the Pathophysiology of Pregnancy and in Systemic Autoimmune Rheumatic Diseases During Pregnancy.Frontiers in immunology · 2020Review
- The new complement inhibitor CRIg/FH ameliorates lupus nephritis in lupus-prone MRL/lpr mice.BMC nephrology · 2019Article
- Atypical hemolytic uremic syndrome: a syndrome in need of clarity.Clinical kidney journal · 2019Article
- Eculizumab prevents thrombotic microangiopathy in patients with atypical haemolytic uraemic syndrome in a long-term observational study.Clinical kidney journal · 2019Article
- Complement Therapeutics in Autoimmune Disease.Frontiers in immunology · 2019Review
- Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.Clinical kidney journal · 2017Article
- Anti-C1q autoantibodies as markers of renal involvement in childhood-onset systemic lupus erythematosus.Pediatric nephrology (Berlin, Germany) · 2017Article
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundTreatment of systemic lupus erythematosus (SLE) with severe diffuse proliferative nephritis is often challenging, particularly in small children in whom a genetic conditioning is likely to play a role. The effectiveness of standard therapy based on glucocorticoid and immunosuppressive drugs is often unsatisfactory. CASE: A 4 year-old girl, whose parents were first-grade cousins of Moroccan ancestry, developed SLE that progressed to severe renal involvement despite standard therapy. She had persistently undetectable serum C4 levels and very low C3 levels (<30 mg/dl), and extremely high anti-DNA titers (>1:640) that remained unmodified during 2 years of follow-up. No mutations of genes encoding for complement inhibitors were detected. Despite aggressive therapy based on prednisone, plasma exchanges, and cyclosporine, the child worsened and eventually developed features of atypical hemolytic uremic syndrome (aHUS). Treatment with eculizumab provided prompt remission of vasculitis, proteinuria, and hematuria, with normalization of renal function. Two attempts to withdraw eculizumab were followed by severe relapses and rescued by reinstating treatment. The child has been treated with eculizumab for > 17 months without relevant side effects.
conclusionC5 inhibition by eculizumab completely reversed clinical symptoms and laboratory renal signs of severe lupus nephritis. Blocking complement-system activation with the use of targeted drugs may be a new and exciting strategy to treat SLE patients unresponsive to conventional therapy.
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