ArticleClinical journal of the American Society of Nephrology : CJASN2012
Factor I autoantibodies in patients with atypical hemolytic uremic syndrome: disease-associated or an epiphenomenon?
Article in Clinical journal of the American Society of Nephrology : CJASN, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 1 of them a synthesis that pooled it.
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Who cites it
31 citing papers in PubMed, 1 synthesis or guideline pooled it, 62 citations in OpenAlex.
- Systematic review of atypical hemolytic uremic syndrome biomarkers.Pediatric nephrology (Berlin, Germany) · 2022Pooled it
- C3 Glomerulonephritis Associated With Anti-complement Factor B Antibodies Following Anti-cancer Treatment With Pembrolizumab.Kidney medicine · 2026Article
- Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.Frontiers in immunology · 2025Review
- Anti-factor B antibodies in atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2024Article
- Observational
- Alternative pathway diagnostics.Immunological reviews · 2023Review
- Diagnosis and treatment of thrombotic microangiopathy.International journal of laboratory hematology · 2022Review
- Thrombotic microangiopathies: An illustrated review.Research and practice in thrombosis and haemostasis · 2022Review
- Thrombotic Microangiopathy, an Unusual Form of Monoclonal Gammopathy of Renal Significance: Report of 3 Cases and Literature Review.Frontiers in immunology · 2021Review
- The Immunopathology of Complement Proteins and Innate Immunity in Autoimmune Disease.Clinical reviews in allergy & immunology · 2020Review
- Differential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome-related factor H mutation.Kidney international · 2019Article
- Genotype-phenotype correlations of low-frequency variants in the complement system in renal disease and age-related macular degeneration.Clinical genetics · 2018Article
- Thrombotic Microangiopathy and the Kidney.Clinical journal of the American Society of Nephrology : CJASN · 2018Review
- Serum complement factor I is associated with disease activity of systemic lupus erythematosus.Oncotarget · 2018Article
- Atypical hemolytic uremic syndrome and eculizumab therapy in children.Korean journal of pediatrics · 2018Review
- Diseases of complement dysregulation-an overview.Seminars in immunopathology · 2018Review
- Interpretation of Serological Complement Biomarkers in Disease.Frontiers in immunology · 2018Review
- Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland.Kidney international · 2017Article
- Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.Clinical kidney journal · 2017Article
- Thrombotic Microangiopathy in Inverted Formin 2Journal of the American Society of Nephrology : JASN · 2017Article
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Authors and funding
15 authors at 5 institutions in 2 countries.
Funding
Abstract
BACKGROUND AND
objectivesAtypical hemolytic uremic syndrome is a disease associated with mutations in the genes encoding the complement regulators factors H and I. In addition, factor H autoantibodies have been reported in ∼10% of patients with atypical hemolytic uremic syndrome. This study searched for the presence of factor I autoantibodies in atypical hemolytic uremic syndrome. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This study screened 175 atypical hemolytic uremic syndrome patients for factor I autoantibodies using ELISA with confirmatory Western blotting. Functional studies using purified immunoglobulin from one patient were subsequently undertaken.
resultsFactor I autoantibodies were detected in three patients. In one patient with a high titer of autoantibody, the titer was tracked over time and was found to have no association with disease activity. This study found evidence of an immune complex of antibody and factor I in this patient, but purified IgG, isolated from current serum samples, had only a minor effect on fluid phase and cell surface complement regulation. Genetic analysis of the three patients with factor I autoantibodies revealed that they had two copies of the genes encoding factor H-related proteins 1 and 3 and therefore, did not have a deletion commonly associated with factor H autoantibodies in atypical hemolytic uremic syndrome. Two patients, however, had functionally significant mutations in complement factor H.
conclusionsThese findings reinforce the concept of multiple concurrent risk factors being associated with atypical hemolytic uremic syndrome but question whether autoantibodies per se predispose to atypical hemolytic uremic syndrome.
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