Schaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N et al (2018) Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int 94(2):408–418. https://doi.org/10.1016/j.kint.2018.02.029

Article  PubMed  Google Scholar 

Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaimé F, Dragon-Durey MA, Ngo S et al (2013) Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 8(4):554–562. https://doi.org/10.2215/cjn.04760512

Article  CAS  PubMed  PubMed Central  Google Scholar 

Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH et al (2015) Atypical aHUS: state of the art. Mol Immunol 67(1):31–42. https://doi.org/10.1016/j.molimm.2015.03.246

Article  CAS  PubMed  Google Scholar 

Afshar-Kharghan V (2016) Atypical hemolytic uremic syndrome. Hematology Am Soc Hematol Educ Program 2016(1):217–225. https://doi.org/10.1182/asheducation-2016.1.217

Article  PubMed  PubMed Central  Google Scholar 

Pivovarova AI, Thongprayoon C, Hansrivijit P, Kaewput W, Qureshi F, Boonpheng B et al (2020) Thrombotic microangiopathy among hospitalized patients with systemic lupus erythematosus in the United States. Dis Cases. https://doi.org/10.3390/diseases9010003

Article  Google Scholar 

Schubart A, Anderson K, Mainolfi N, Sellner H, Ehara T, Adams CM et al (2019) Small-molecule factor B inhibitor for the treatment of complement-mediated diseases. Proc Natl Acad Sci U S A 116(16):7926–7931. https://doi.org/10.1073/pnas.1820892116

Article  CAS  PubMed  PubMed Central  Google Scholar 

Wong EKS, Praga M, Nester C (2021) Iptacopan (LNP023): a novel oral complement alternative pathway factor b inhibitor safely and effectively stabilises eGFR in C3 glomerulopathy. Nephrol Dial Transplant 36(Suppl 1):i25. https://doi.org/10.1681/ASN.20203110S155c

Article  Google Scholar 

Risitano AM, Röth A, Soret J, Frieri C, de Fontbrune FS, Marano L et al (2021) Addition of iptacopan, an oral factor B inhibitor, to eculizumab in patients with paroxysmal nocturnal haemoglobinuria and active haemolysis: an open-label, single-arm, phase 2, proof-of-concept trial. Lancet Haematol. https://doi.org/10.1016/s2352-3026(21)00028-4

Article  PubMed  Google Scholar 

Kavanagh D, Greenbaum LA, Bagga A, Karki RG, Chen CW, Vasudevan S et al (2023) Design and rationale of the APPELHUS phase 3 open-label study of factor B inhibitor iptacopan for atypical hemolytic uremic syndrome. Kidney Int Rep 8(7):1332–1341. https://doi.org/10.1016/j.ekir.2023.04.029

Article  PubMed  PubMed Central  Google Scholar 

Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC et al (2024) EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis 83(1):15–29. https://doi.org/10.1136/ard-2023-224762

Article  CAS  PubMed  Google Scholar 

Wright RD, Bannerman F, Beresford MW, Oni L (2020) A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy. BMC Nephrol 21(1):245. https://doi.org/10.1186/s12882-020-01888-5

Article  CAS  PubMed  PubMed Central  Google Scholar 

Martis N, Jamme M, Bagnis-Isnard C, Pouteil-Noble C, Presne C, Vigneau C et al (2021) Systemic autoimmune disorders associated with thrombotic microangiopathy: a cross-sectional analysis from the French national TMA registry: systemic autoimmune disease-associated TMA. Eur J Intern Med 93:78–86. https://doi.org/10.1016/j.ejim.2021.05.040

Article  CAS  PubMed  Google Scholar 

Fakhouri F, Schwotzer N, Frémeaux-Bacchi V (2023) How i diagnose and treat atypical hemolytic uremic syndrome. Blood 141(9):984–995. https://doi.org/10.1182/blood.2022017860

Article  CAS  PubMed  Google Scholar 

Brocklebank V, Walsh PR, Smith-Jackson K, Hallam TM, Marchbank KJ, Wilson V et al (2023) Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study. Blood 142(16):1371–1386. https://doi.org/10.1182/blood.2022018833

Article  CAS  PubMed  PubMed Central  Google Scholar 

Nishimura J, Yamamoto M, Hayashi S, Ohyashiki K, Ando K, Brodsky AL et al (2014) Genetic variants in C5 and poor response to eculizumab. N Engl J Med 370(7):632–639. https://doi.org/10.1056/nejmoa1311084

Article  CAS  PubMed  Google Scholar