{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T20:43:42Z","timestamp":1793133822844,"version":"4.5.0"},"reference-count":38,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,8,23]],"date-time":"2022-08-23T00:00:00Z","timestamp":1661212800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Atypical hemolytic uremic syndrome (aHUS) an important form of a thrombotic microangiopathy (TMA) that can frequently lead to acute kidney injury (AKI). An important subset of aHUS is the anti-factor H associated aHUS. This variant of aHUS can occur due to deletion of the complement factor H genes, <jats:italic>CFHR1<\/jats:italic> and <jats:italic>CFHR3<\/jats:italic>, along with the presence of anti-factor H antibodies. However, it is a point of interest to note that not all patients with anti-factor H associated aHUS have a <jats:italic>CFHR1\/R3<\/jats:italic> deletion. Factor-H has a vital role in the regulation of the complement system, specifically the alternate pathway. Therefore, dysregulation of the complement system can lead to inflammatory or autoimmune diseases. Patients with this disease respond well to treatment with plasma exchange therapy along with Eculizumab and immunosuppressant therapy. Anti-factor H antibody associated aHUS has a certain genetic predilection therefore there is focus on further advancements in the diagnosis and management of this disease. In this article we discuss the baseline characteristics of patients with anti-factor H associated aHUS, their triggers, various treatment modalities and future perspectives.<\/jats:p>","DOI":"10.3389\/fimmu.2022.931210","type":"journal-article","created":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T03:26:37Z","timestamp":1661311597000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":32,"title":["Anti-factor H antibody and its role in atypical hemolytic uremic syndrome"],"prefix":"10.3389","volume":"13","author":[{"given":"Rupesh","family":"Raina","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guneive","family":"Mangat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gordon","family":"Hong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raghav","family":"Shah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikhil","family":"Nair","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian","family":"Abboud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sumedha","family":"Bagga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sidharth Kumar","family":"Sethi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,8,23]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.2478\/prilozi-2021-0029","article-title":"Anti-factor h antibody-associated atypical hemolytic uremic syndrome: A case report","volume":"42","author":"Abazi-Emini","year":"2021","journal-title":"Prilozi (Makedonska akademija na naukite i umetnostite Oddelenie za medicinski nauki)"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/6904858","article-title":"Anti-factor h antibodies in Egyptian children with hemolytic uremic syndrome","volume":"2021","author":"Shawky","year":"2021","journal-title":"Int J Nephrol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.831044","article-title":"Editorial: Function and dysfunction of complement factor h","volume":"12","author":"J\u00f3zsi","year":"2022","journal-title":"Front Immunol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.607211","article-title":"Factor h autoantibodies and complement-mediated diseases","volume":"11","author":"Zhang","year":"2020","journal-title":"Front Immunol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.660382","article-title":"Autoantibodies against the complement regulator factor h in the serum of patients with neuromyelitis optica spectrum disorder","volume":"12","author":"Uzonyi","year":"2021","journal-title":"Front Immunol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.602284","article-title":"Functional characterization of rare genetic variants in the n-terminus of complement factor h in aHUS, C3G, and AMD","volume":"11","author":"Wong","year":"2021","journal-title":"Front Immunol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-018-4074-4","article-title":"Immunological features and functional analysis of anti-CFH autoantibodies in patients with atypical hemolytic uremic syndrome","volume":"34","author":"Guo","year":"2019","journal-title":"Pediatr Nephrol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1055\/s-0034-1375297","article-title":"Complement factor h-antibody-associated hemolytic uremic syndrome: pathogenesis, clinical presentation, and treatment","volume":"40","author":"Hofer","year":"2014","journal-title":"Semin Thromb Hemost"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-05-221549","article-title":"Association of factor h autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome","volume":"115","author":"Moore","year":"2010","journal-title":"Blood"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.01260212","article-title":"Complement factor h-related protein 1 deficiency and factor h antibodies in pediatric patients with atypical hemolytic uremic syndrome","volume":"8","author":"Hofer","year":"2013","journal-title":"Clin J Am Soc Nephrol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01282","article-title":"Clinical and immunological profile of anti-factor h antibody associated atypical hemolytic uremic syndrome: A nationwide database","volume":"10","author":"Puraswani","year":"2019","journal-title":"Front Immunol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2018.02.029","article-title":"Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome","volume":"94","author":"Schaefer","year":"2018","journal-title":"Kidney Int"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-021-05390-4","article-title":"Anti-factor h antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection","volume":"37","author":"Khandelwal","year":"2022","journal-title":"Pediatr Nephrol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2006080811","article-title":"Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome","volume":"18","author":"Sellier-Leclerc","year":"2007","journal-title":"J Am Soc Nephrol: JASN"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00853","article-title":"Rare functional variants in complement genes and anti-FH autoantibodies-associated aHUS","volume":"10","author":"Valoti","year":"2019","journal-title":"Front Immunol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1111\/ped.12549","article-title":"Atypical hemolytic uremic syndrome: Korean pediatric series","volume":"57","author":"Lee","year":"2015","journal-title":"Pediatr International: Off J Japan Pediatr Soc"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.04760512","article-title":"Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults","volume":"8","author":"Fremeaux-Bacchi","year":"2013","journal-title":"Clin J Am Soc Nephrol: CJASN"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2017.04.028","article-title":"Factor h autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland","volume":"92","author":"Brocklebank","year":"2017","journal-title":"Kidney Int"},{"key":"B19","article-title":"Anti-compliment factor h antibody associated hemolytic uremic syndrome in children with abbreviated plasma exchanges: A 12-month follow-up study","volume":"15","author":"Tiewsoh","year":"2021","journal-title":"Iran J Kidney Dis"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-016-3562-7","article-title":"The clinical and laboratory features of Chinese han anti-factor h autoantibody-associated hemolytic uremic syndrome","volume":"32","author":"Song","year":"2017","journal-title":"Pediatr Nephrol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/j.molimm.2015.12.005","article-title":"Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor h autoantibodies in atypical hemolytic uremic syndrome","volume":"70","author":"Nozal","year":"2016","journal-title":"Mol Immunol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.02210310","article-title":"Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype","volume":"5","author":"Noris","year":"2010","journal-title":"Clin J Am Soc Nephrol: CJASN"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2010030315","article-title":"Clinical features of anti-factor h autoantibody-associated hemolytic uremic syndrome","volume":"21","author":"Dragon-Durey","year":"2010","journal-title":"J Am Soc Nephrol: JASN"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-09-109876","article-title":"Factor h autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1\/CFHR3 deficiency","volume":"111","author":"J\u00f3zsi","year":"2008","journal-title":"Blood"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2005.030783","article-title":"The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor h and membrane cofactor protein: Evidence from two independent cohorts","volume":"42","author":"Fremeaux-Bacchi","year":"2005","journal-title":"J Med Genet"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-02-071472","article-title":"Anti factor h autoantibodies block c-terminal recognition function of factor h in hemolytic uremic syndrome","volume":"110","author":"J\u00f3zsi","year":"2007","journal-title":"Blood"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1111\/imm.12916","article-title":"Characterization of genetic predisposition and autoantibody profile in atypical haemolytic-uraemic syndrome","volume":"154","author":"Gurjar","year":"2018","journal-title":"Immunology"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-014-2948-7","article-title":"Effect of plasma exchange and immunosuppressive medications on antibody titers and outcome in anti-complement factor h antibody-associated hemolytic uremic syndrome","volume":"30","author":"Khandelwal","year":"2015","journal-title":"Pediatr Nephrol"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1002\/jca.21470","article-title":"Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the writing committee of the American society for apheresis: The seventh special issue","volume":"31","author":"Schwartz","year":"2016","journal-title":"J Clin Apher"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1111\/joim.1254","article-title":"Haemolytic uraemic syndrome","volume":"281","author":"Karpman","year":"2017","journal-title":"J Internal Med"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-018-4091-3","article-title":"Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use","volume":"34","author":"Wijnsma","year":"2019","journal-title":"Pediatr Nephrol"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-6-60","article-title":"Atypical hemolytic uremic syndrome","volume":"6","author":"Loirat","year":"2011","journal-title":"Orphanet J Rare Dis"},{"key":"B33","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1093\/qjmed\/hcv082","article-title":"A national specialized service in England for atypical haemolytic uraemic syndrome-the first year\u2019s experience","volume":"109","author":"Sheerin","year":"2016","journal-title":"QJM: Monthly J Assoc Physicians"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1111\/1744-9987.12763","article-title":"Atypical hemolytic-uremic syndrome: An update on pathophysiology, diagnosis and treatment","volume":"23","author":"Raina","year":"2018","journal-title":"Ther Apheresis Dialysis"},{"key":"B35","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1016\/j.kint.2015.11.026","article-title":"Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome","volume":"89","author":"Greenbaum","year":"2016","journal-title":"Kidney Int"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-013-2654-x","article-title":"Successful treatment of DEAP-HUS with eculizumab","volume":"29","author":"Noone","year":"2014","journal-title":"Pediatr Nephrol"},{"key":"B37","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1007\/s00467-015-3076-8","article-title":"An international consensus approach to the management of atypical hemolytic uremic syndrome in children","volume":"31","author":"Loirat","year":"2016","journal-title":"Pediatr Nephrol"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12273","article-title":"Outcomes of renal transplant in patients with anti-complement factor h antibody-associated hemolytic uremic syndrome","volume":"18","author":"Khandelwal","year":"2014","journal-title":"Pediatr Transplant"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2022.931210\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T03:26:41Z","timestamp":1661311601000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2022.931210\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,23]]},"references-count":38,"alternative-id":["10.3389\/fimmu.2022.931210"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2022.931210","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,23]]},"article-number":"931210"}}