ArticleKidney international2017
Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland.
Article in Kidney international, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers, 3 of them syntheses that pooled it.
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Who cites it
41 citing papers in PubMed, 3 syntheses or guidelines pooled it, 67 citations in OpenAlex.
- Comparison between effectiveness and safety of plasma therapy and complement inhibitors for the treatment of atypical hemolytic uremic syndrome: a systematic review and meta-analysis of real-world data.Pediatric nephrology (Berlin, Germany) · 2026Pooled it
- Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach From the Writing Committee of the American Society for Apheresis: The Tenth Special Issue.Journal of clinical apheresis · 2026Guideline
- Variants in complement genes are uncommon in patients with anti-factor H autoantibody-associated atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2023Pooled it
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Renal-Limited Thrombotic Microangiopathy in Infants: A Case Series.Kidney medicine · 2026Article
- Clinical and cost-effectiveness of eculizumab withdrawal in atypical haemolytic uraemic syndrome: the SETS aHUS multi-centre, open-label, prospective and single-arm study.Health technology assessment (Winchester, England) · 2026Article
- Diagnostic and therapeutic challenges into snakebite-induced thrombotic microangiopathy: a case report and review of the literature.Journal of medical case reports · 2026Review
- Eculizumab withdrawal and monitoring in atypical haemolytic uraemic syndrome (SETS aHUS): a multicentre, open label, prospective, single arm trial.The Lancet regional health. Europe · 2025Article
- Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.Journal of clinical medicine · 2025Review
- Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.Frontiers in immunology · 2025Review
- Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum.Kidney international · 2024Article
- Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus.Pediatric nephrology (Berlin, Germany) · 2024Article
- Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?Clinical and experimental nephrology · 2024Article
- Primary Thrombotic Microangiopathy in Pediatric Patients.Global pediatric health · 2024Article
- Observational
- Factor H autoantibodies contribute to complement dysregulation in multisystem inflammatory syndrome in children (MIS-C).American journal of hematology · 2023Article
- Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management.Journal of multidisciplinary healthcare · 2023Review
- The loss of glycocalyx integrity impairs complement factor H binding and contributes to cyclosporine-induced endothelial cell injury.Frontiers in medicine · 2023Article
- Clinical features of children with anti-CFH autoantibody-associated hemolytic uremic syndrome: a report of 8 cases.Renal failure · 2022Article
- Diagnosis and treatment of thrombotic microangiopathy.International journal of laboratory hematology · 2022Review
Corrections and comments
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Authors and funding
24 authors at 13 institutions in 2 countries.
Funding
Abstract
Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort analysis. Here we examined 175 children presenting with atypical hemolytic uremic syndrome in the United Kingdom and Ireland for factor H autoantibodies that included 17 children with titers above the international standard. Of the 17, seven had a concomitant rare genetic variant in a gene encoding a complement pathway component or regulator. Two children received supportive treatment; both developed established renal failure. Plasma exchange was associated with a poor rate of renal recovery in seven of 11 treated. Six patients treated with eculizumab recovered renal function. Contrary to global practice, immunosuppressive therapy to prevent relapse in plasma exchange-treated patients was not adopted due to concerns over treatment-associated complications. Without immunosuppression, the relapse rate was high (five of seven). However, reintroduction of treatment resulted in recovery of renal function. All patients treated with eculizumab achieved sustained remission. Five patients received renal transplants without specific factor H autoantibody-targeted treatment with recurrence in one who also had a functionally significant CFI mutation. Thus, our current practice is to initiate eculizumab therapy for treatment of factor H autoantibody-mediated atypical hemolytic uremic syndrome rather than plasma exchange with or without immunosuppression. Based on this retrospective analysis we see no suggestion of inferior treatment, albeit the strength of our conclusions is limited by the small sample size.
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