{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T20:41:10Z","timestamp":1792442470685,"version":"4.5.0"},"reference-count":96,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T00:00:00Z","timestamp":1792368000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T00:00:00Z","timestamp":1792368000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Nephrol"],"DOI":"10.1007\/s00467-026-07571-5","type":"journal-article","created":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T07:16:45Z","timestamp":1792394205000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Therapy for kidney vasculitis in children during the acute phase and maintenance: updates and insights from new adult therapies"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8311-5409","authenticated-orcid":false,"given":"Andrea","family":"Angioi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doloretta","family":"Piras","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Lepori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonello","family":"Pani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,19]]},"reference":[{"key":"7571_CR1","doi-asserted-by":"publisher","first-page":"1197","DOI":"10.1016\/S0140-6736(02)11279-7","volume":"360","author":"JMM Gardner-Medwin","year":"2002","unstructured":"Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Sch\u00f6nlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197\u20131202","journal-title":"Lancet"},{"key":"7571_CR2","doi-asserted-by":"publisher","first-page":"6621","DOI":"10.3390\/jcm13216621","volume":"13","author":"S Casta\u00f1eda","year":"2024","unstructured":"Casta\u00f1eda S, Quiroga-Colina P, Floranes P et al (2024) IgA Vasculitis (Henoch\u2013Sch\u00f6nlein Purpura): an update on treatment. J Clin Med 13:6621. https:\/\/doi.org\/10.3390\/jcm13216621","journal-title":"J Clin Med"},{"key":"7571_CR3","doi-asserted-by":"publisher","DOI":"10.1186\/cc13083","volume":"17","author":"M Duffett","year":"2013","unstructured":"Duffett M, Choong K, Hartling L, Menon K, Thabane L, Cook DJ (2013) Randomized controlled trials in pediatric critical care: a scoping review. Crit Care 17:R256","journal-title":"Crit Care"},{"key":"7571_CR4","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1681\/ASN.0000000888","volume":"37","author":"L Oni","year":"2026","unstructured":"Oni L, Smoyer WE, Denburg M et al (2026) The use of extrapolation to promote clinical trials in pediatric nephrology. J Am Soc Nephrol 37:616\u2013618. https:\/\/doi.org\/10.1681\/ASN.0000000888","journal-title":"J Am Soc Nephrol"},{"key":"7571_CR5","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1186\/s12969-025-01149-5","volume":"23","author":"H Ercan Emreol","year":"2025","unstructured":"Ercan Emreol H, Yildirim-Toruner C, Jelusic M, Twilt M, Ozen S (2025) New avenues in childhood vasculitis. Pediatr Rheumatol Online J 23:97","journal-title":"Pediatr Rheumatol Online J"},{"key":"7571_CR6","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1007\/s00467-024-06502-6","volume":"40","author":"M Vivarelli","year":"2025","unstructured":"Vivarelli M, Samuel S, Coppo R et al (2025) IPNA clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis. Pediatr Nephrol 40:533\u2013569","journal-title":"Pediatr Nephrol"},{"key":"7571_CR7","doi-asserted-by":"publisher","first-page":"340","DOI":"10.1016\/j.kint.2019.08.022","volume":"97","author":"Y Makita","year":"2020","unstructured":"Makita Y, Suzuki H, Kano T et al (2020) TLR9 activation induces aberrant IgA glycosylation via APRIL- and IL-6\u2013mediated pathways in IgA nephropathy. Kidney Int 97:340\u2013349","journal-title":"Kidney Int"},{"key":"7571_CR8","doi-asserted-by":"publisher","first-page":"1765","DOI":"10.1093\/ndt\/gfaa344","volume":"36","author":"F Sallustio","year":"2021","unstructured":"Sallustio F, Curci C, Chaoul N et al (2021) High levels of gut-homing immunoglobulin A+ B lymphocytes support the pathogenic role of intestinal mucosal hyperresponsiveness in immunoglobulin A nephropathy patients. Nephrol Dial Transplant 36:1765","journal-title":"Nephrol Dial Transplant"},{"key":"7571_CR9","doi-asserted-by":"publisher","first-page":"1795","DOI":"10.1681\/ASN.2011050464","volume":"22","author":"H Suzuki","year":"2011","unstructured":"Suzuki H, Kiryluk K, Novak J et al (2011) The pathophysiology of IgA nephropathy. J Am Soc Nephrol 22:1795\u20131803","journal-title":"J Am Soc Nephrol"},{"key":"7571_CR10","doi-asserted-by":"publisher","first-page":"2465","DOI":"10.3390\/jcm13092465","volume":"13","author":"DS Chiarenza","year":"2024","unstructured":"Chiarenza DS, Verrina EE, La Porta E et al (2024) Biologics and non-biologics immunosuppressive treatments for IgA nephropathy in both adults and children. J Clin Med 13:2465","journal-title":"J Clin Med"},{"key":"7571_CR11","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1016\/S0140-6736(23)01554-4","volume":"402","author":"R Lafayette","year":"2023","unstructured":"Lafayette R, Kristensen J, Stone A, Floege J, Tesa\u0159 V, Trimarchi H, Zhang H, Eren N, Paliege A, Reich HN, Rovin BH, Barratt J NefIgArd Trial Investigators (2023) Efficacy and safety of a targeted-release formulation of budesonide in patients with primary IgA nephropathy (NefIgArd): 2-year results from a randomised phase 3 trial. Lancet 402:859\u2013870. https:\/\/doi.org\/10.1016\/S0140-6736(23)01554-4.","journal-title":"Lancet"},{"key":"7571_CR12","doi-asserted-by":"publisher","first-page":"679","DOI":"10.1007\/s00281-021-00882-9","volume":"43","author":"NR Medjeral-Thomas","year":"2021","unstructured":"Medjeral-Thomas NR, Cook HT, Pickering MC (2021) Complement activation in IgA nephropathy. Semin Immunopathol 43:679\u2013690","journal-title":"Semin Immunopathol"},{"key":"7571_CR13","doi-asserted-by":"publisher","first-page":"1475","DOI":"10.1007\/s40265-023-01940-2","volume":"83","author":"A Rajasekaran","year":"2023","unstructured":"Rajasekaran A, Green TJ, Renfrow MB, Julian BA, Novak J, Rizk DV (2023) Current understanding of complement proteins as therapeutic targets for the treatment of immunoglobulin A nephropathy. Drugs 83:1475\u20131499","journal-title":"Drugs"},{"key":"7571_CR14","doi-asserted-by":"publisher","first-page":"4405","DOI":"10.3390\/jcm10194405","volume":"10","author":"M Mizerska-Wasiak","year":"2021","unstructured":"Mizerska-Wasiak M, Such-Gruchot A, Cicho\u0144-Kawa K et al (2021) The role of complement component C3 activation in the clinical presentation and prognosis of IgA nephropathy\u2013-a national study in children. J Clin Med 10:4405","journal-title":"J Clin Med"},{"key":"7571_CR15","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1007\/s00467-019-04363-y","volume":"35","author":"JD Delbet","year":"2020","unstructured":"Delbet JD, Geslain G, Auger M, Hogan J, Salomon R, Peuchmaur M, Desch\u00eanes G, Buob D, Parmentier C, Ulinski T (2020) Histological prognostic factors in children with Henoch-Sch\u00f6nlein purpura nephritis. Pediatr Nephrol 35:313\u2013320. https:\/\/doi.org\/10.1007\/s00467-019-04363-y","journal-title":"Pediatr Nephrol"},{"key":"7571_CR16","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1042\/cs0830281","volume":"83","author":"DJ O\u2019Donoghue","year":"1992","unstructured":"O\u2019Donoghue DJ, Jewkes F, Postlethwaite RJ, Ballardie FW (1992) Autoimmunity to glomerular antigens in Henoch-Schoenlein nephritis. Clin Sci 83:281\u2013287","journal-title":"Clin Sci"},{"key":"7571_CR17","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2022.957660","volume":"13","author":"S Ge","year":"2022","unstructured":"Ge S, Zhu X, Xu Q et al (2022) Neutrophils in ANCA-associated vasculitis: mechanisms and implications for management. Front Pharmacol 13:957660","journal-title":"Front Pharmacol"},{"key":"7571_CR18","doi-asserted-by":"publisher","first-page":"i139","DOI":"10.1093\/ndt\/gfw299","volume":"32","author":"S Sethi","year":"2017","unstructured":"Sethi S, Zand L, De Vriese AS et al (2017) Complement activation in pauci-immune necrotizing and crescentic glomerulonephritis: results of a proteomic analysis. Nephrol Dial Transplant 32:i139\u2013i145","journal-title":"Nephrol Dial Transplant"},{"key":"7571_CR19","doi-asserted-by":"publisher","first-page":"915","DOI":"10.3390\/cells9040915","volume":"9","author":"E Fousert","year":"2020","unstructured":"Fousert E, Toes R, Desai J (2020) Neutrophil extracellular traps (NETs) take the central stage in driving autoimmune responses. Cells 9:915","journal-title":"Cells"},{"key":"7571_CR20","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1681\/ASN.2008050497","volume":"20","author":"A Schreiber","year":"2009","unstructured":"Schreiber A, Xiao H, Jennette JC, Schneider W, Luft FC, Kettritz R (2009) C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis. J Am Soc Nephrol 20:289\u2013298","journal-title":"J Am Soc Nephrol"},{"key":"7571_CR21","doi-asserted-by":"publisher","first-page":"1687","DOI":"10.1136\/annrheumdis-2015-207601","volume":"75","author":"C Rahmattulla","year":"2016","unstructured":"Rahmattulla C, Mooyaart AL, van Hooven D et al (2016) Genetic variants in ANCA-associated vasculitis: a meta-analysis. Ann Rheum Dis 75:1687\u20131692","journal-title":"Ann Rheum Dis"},{"key":"7571_CR22","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.991256","volume":"13","author":"W-M Zhao","year":"2022","unstructured":"Zhao W-M, Wang Z-J, Shi R et al (2022) Environmental factors influencing the risk of ANCA-associated vasculitis. Front Immunol 13:991256","journal-title":"Front Immunol"},{"key":"7571_CR23","first-page":"975","volume":"41","author":"C Marvisi","year":"2023","unstructured":"Marvisi C, Ricordi C, Galli E et al (2023) Pros and cons of TNF inhibitors and tocilizumab in the treatment of large-vessel vasculitis. Clin Exp Rheumatol 41:975\u2013981","journal-title":"Clin Exp Rheumatol"},{"key":"7571_CR24","doi-asserted-by":"publisher","first-page":"SI122","DOI":"10.1093\/rheumatology\/kead693","volume":"63","author":"U Kaya Akca","year":"2024","unstructured":"Kaya Akca U, Batu ED, Jelusic M et al (2024) Comparison of EULAR\/PRINTO\/PReS Ankara 2008 and 2022 ACR\/EULAR classification criteria for granulomatosis with polyangiitis in children. Rheumatology 63:SI122\u2013SI128","journal-title":"Rheumatology"},{"key":"7571_CR25","doi-asserted-by":"publisher","first-page":"936","DOI":"10.1136\/ard.2005.046300","volume":"65","author":"S Ozen","year":"2006","unstructured":"Ozen S, Ruperto N, Dillon MJ et al (2006) EULAR\/PReS endorsed consensus criteria \\* for the classification of childhood vasculitides. Ann Rheum Dis 65:936\u2013941","journal-title":"Ann Rheum Dis"},{"key":"7571_CR26","doi-asserted-by":"publisher","first-page":"1299","DOI":"10.1093\/ndt\/gfae009","volume":"39","author":"K Rohner","year":"2024","unstructured":"Rohner K, Marlais M, Ahn YH et al (2024) Outcome of immunosuppression in children with IgA vasculitis\u2013related nephritis. Nephrol Dial Transplant 39:1299\u20131309","journal-title":"Nephrol Dial Transplant"},{"key":"7571_CR27","doi-asserted-by":"publisher","first-page":"S1","DOI":"10.1016\/j.kint.2025.04.004","volume":"108","author":"BH Rovin","year":"2025","unstructured":"Rovin BH, Barratt J et al Kidney Disease: Improving Global Outcomes (KDIGO) IgAN and IgAV Work Group (2025) KDIGO 2025 clinical practice guideline for the management of immunoglobulin A nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV). Kidney Int 108:S1\u2013S71","journal-title":"Kidney Int"},{"key":"7571_CR28","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1136\/bmj.2.6078.11","volume":"2","author":"R Counahan","year":"1977","unstructured":"Counahan R, Winterborn MH, White RH et al (1977) Prognosis of Henoch-Sch\u00f6nlein nephritis in children. Br Med J 2:11\u201314","journal-title":"Br Med J"},{"key":"7571_CR29","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1007\/s004670050446","volume":"12","author":"P Niaudet","year":"1998","unstructured":"Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Sch\u00f6nlein-Henoch purpura nephritis. Pediatr Nephrol 12:238\u2013243","journal-title":"Pediatr Nephrol"},{"key":"7571_CR30","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1007\/s00467-003-1315-x","volume":"19","author":"P Tarshish","year":"2004","unstructured":"Tarshish P, Bernstein J, Edelmann CM (2004) Henoch-Sch\u00f6nlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19:51\u201356","journal-title":"Pediatr Nephrol"},{"key":"7571_CR31","doi-asserted-by":"publisher","first-page":"2159","DOI":"10.1007\/s00467-011-1919-5","volume":"26","author":"O Jauhola","year":"2011","unstructured":"Jauhola O, Ronkainen J, Autio-Harmainen H et al (2011) Cyclosporine A vs. methylprednisolone for Henoch-Sch\u00f6nlein nephritis: a randomized trial. Pediatr Nephrol 26:2159\u20132166","journal-title":"Pediatr Nephrol"},{"key":"7571_CR32","doi-asserted-by":"publisher","first-page":"2333","DOI":"10.12659\/MSM.904206","volume":"23","author":"Z Lu","year":"2017","unstructured":"Lu Z, Song J, Mao J, Xia Y, Wang C (2017) Evaluation of mycophenolate mofetil and low-dose steroid combined therapy in moderately severe Henoch-Sch\u00f6nlein purpura nephritis. Med Sci Monit 23:2333\u20132339","journal-title":"Med Sci Monit"},{"key":"7571_CR33","doi-asserted-by":"publisher","first-page":"765","DOI":"10.1007\/s00467-011-2057-9","volume":"27","author":"Y Du","year":"2012","unstructured":"Du Y, Hou L, Zhao C, Han M, Wu Y (2012) Treatment of children with Henoch-Sch\u00f6nlein purpura nephritis with mycophenolate mofetil. Pediatr Nephrol 27:765\u2013771","journal-title":"Pediatr Nephrol"},{"key":"7571_CR34","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1007\/s00467-017-3846-6","volume":"33","author":"A Hackl","year":"2018","unstructured":"Hackl A, Becker JU, K\u00f6rner LM et al (2018) Mycophenolate mofetil following glucocorticoid treatment in Henoch-Sch\u00f6nlein purpura nephritis: the role of early initiation and therapeutic drug monitoring. Pediatr Nephrol 33:619\u2013629","journal-title":"Pediatr Nephrol"},{"key":"7571_CR35","unstructured":"Ding G (2025) Clinical study on the efficacy and safety of telitacicept in the treatment of pediatric IgA nephropathy or IgA Vasculitis Nephritis [Internet]. clinicaltrials.gov, [cited 7 Feb 2026]. Available from: https:\/\/clinicaltrials.gov\/study\/NCT07052981"},{"key":"7571_CR36","unstructured":"Vera Therapeutics, Inc (2025) A Phase 2b\/3, Multi-part, Randomized, Double-Blinded, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Atacicept in Subjects With IgA Nephropathy (IgAN) [Internet]. clinicaltrials.gov [cited 27 Dec 2025]. Available from: https:\/\/clinicaltrials.gov\/study\/NCT04716231"},{"key":"7571_CR37","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1056\/NEJMoa2305635","volume":"390","author":"M Mathur","year":"2024","unstructured":"Mathur M, Barratt J, Chacko B et al (2024) A phase 2 trial of sibeprenlimab in patients with IgA nephropathy. N Engl J Med 390:20\u201331","journal-title":"N Engl J Med"},{"key":"7571_CR38","doi-asserted-by":"publisher","first-page":"645","DOI":"10.1681\/ASN.0000000534","volume":"36","author":"R Lafayette","year":"2025","unstructured":"Lafayette R, Tumlin J, Fenoglio R et al (2025) Efficacy and safety of ravulizumab in IgA nephropathy: a phase 2 randomized double-blind placebo-controlled trial. J Am Soc Nephrol 36:645\u2013656","journal-title":"J Am Soc Nephrol"},{"key":"7571_CR39","unstructured":"Novartis Pharmaceuticals (2025) A Multi-center, Randomized, Double-blind, placebo-controlled, parallel group, phase III study to evaluate the efficacy and safety of LNP023 in primary IgA nephropathy patients [Internet]. clinicaltrials.gov [cited 27 Dec 2025]. https:\/\/clinicaltrials.gov\/study\/NCT04578834"},{"key":"7571_CR40","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1056\/NEJMoa2410316","volume":"392","author":"V Perkovic","year":"2025","unstructured":"Perkovic V, Barratt J, Rovin B et al (2025) Alternative complement pathway inhibition with iptacopan in IgA nephropathy. N Engl J Med 392:531\u2013543","journal-title":"N Engl J Med"},{"key":"7571_CR41","unstructured":"Huang H (2021) A Study of CD19\/BCMA chimeric antigen receptor T cells therapy for patients with refractory immune nephritis [Internet]. clinicaltrials.gov [cited 2025 Dec 27]. Available from: https:\/\/clinicaltrials.gov\/study\/NCT05085418"},{"key":"7571_CR42","doi-asserted-by":"publisher","first-page":"2077","DOI":"10.1016\/S0140-6736(23)02302-4","volume":"402","author":"BH Rovin","year":"2023","unstructured":"Rovin BH, Barratt J, Heerspink HJL et al (2023) Efficacy and safety of sparsentan versus irbesartan in patients with IgA nephropathy (PROTECT): 2-year results from a randomised, active-controlled, phase 3 trial. Lancet 402:2077\u20132090","journal-title":"Lancet"},{"key":"7571_CR43","doi-asserted-by":"publisher","first-page":"e004481","DOI":"10.1136\/rmdopen-2024-004481","volume":"10","author":"C Barnini","year":"2024","unstructured":"Barnini C, Oni L, Kronbichler A (2024) Course of paediatric ANCA-associated glomerulonephritis: advocating for an age-inclusive approach. RMD Open 10:e004481","journal-title":"RMD Open"},{"key":"7571_CR44","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2025.103824","volume":"24","author":"C Triaille","year":"2025","unstructured":"Triaille C, Terrier B, Hadchouel A, Haddad E, Vaglio A, Fr\u00e9mond M-L (2025) The emerging concept of ANCA-associated vasculitis related to inborn errors of immunity. Autoimmun Rev 24:103824","journal-title":"Autoimmun Rev"},{"key":"7571_CR45","doi-asserted-by":"publisher","first-page":"6065","DOI":"10.3390\/ijms26136065","volume":"26","author":"KA Lisowska","year":"2025","unstructured":"Lisowska KA, Wardowska A (2025) Immunopathology of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Int J Mol Sci 26:6065","journal-title":"Int J Mol Sci"},{"key":"7571_CR46","doi-asserted-by":"publisher","first-page":"1628","DOI":"10.1136\/annrheumdis-2012-202111","volume":"72","author":"P Dolezalova","year":"2013","unstructured":"Dolezalova P, Price-Kuehne FE, \u00d6zen S et al (2013) Disease activity assessment in childhood vasculitis: development and preliminary validation of the Paediatric Vasculitis Activity Score (PVAS). Ann Rheum Dis 72:1628\u20131633","journal-title":"Ann Rheum Dis"},{"key":"7571_CR47","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1136\/annrheumdis-2014-eular.5893","volume":"73","author":"P Dolezalova","year":"2014","unstructured":"Dolezalova P, Wilkinson N, Brogan PA et al (2014) SAT0286 paediatric vasculitis damage index: a new tool for standardised disease assessment. Ann Rheum Dis 73:696\u2013697","journal-title":"Ann Rheum Dis"},{"key":"7571_CR48","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1080\/03009742.2021.2001929","volume":"52","author":"M G\u00e9rard","year":"2023","unstructured":"G\u00e9rard M, de Boysson H, Morello R et al (2023) Early infectious risk in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis according to remission-induction therapy. Scand J Rheumatol 52:161\u2013173","journal-title":"Scand J Rheumatol"},{"key":"7571_CR49","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1056\/NEJMoa0909905","volume":"363","author":"JH Stone","year":"2010","unstructured":"Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221\u2013232","journal-title":"N Engl J Med"},{"key":"7571_CR50","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1056\/NEJMoa0909169","volume":"363","author":"RB Jones","year":"2010","unstructured":"Jones RB, Tervaert JWC, Hauser T et al (2010) Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 363:211\u2013220","journal-title":"N Engl J Med"},{"key":"7571_CR51","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1002\/acr.25455","volume":"77","author":"SJ Gagne","year":"2025","unstructured":"Gagne SJ, Sivaraman V, Bosman ES et al (2025) Comparing rituximab and cyclophosphamide in induction therapy for childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis: an ARChiVe registry cohort study. Arthritis Care Res 77:504\u2013512","journal-title":"Arthritis Care Res"},{"key":"7571_CR52","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1056\/NEJMoa1803537","volume":"382","author":"M Walsh","year":"2020","unstructured":"Walsh M, Merkel PA, Peh C-A et al (2020) Plasma Exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med 382:622\u2013631","journal-title":"N Engl J Med"},{"key":"7571_CR53","doi-asserted-by":"publisher","first-page":"e083277","DOI":"10.1136\/bmjopen-2023-083277","volume":"14","author":"DP McGovern","year":"2024","unstructured":"McGovern DP, McClure ME, Coates M et al (2024) Study protocol for a randomised, phase II, double-blind, experimental medicine study of obinutuzumab versus rituximab in ANCA-associated vasculitis: ObiVas. BMJ Open 14:e083277","journal-title":"BMJ Open"},{"key":"7571_CR54","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","volume":"384","author":"DRW Jayne","year":"2021","unstructured":"Jayne DRW, Merkel PA, Schall TJ, Bekker P (2021) Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med 384:599\u2013609","journal-title":"N Engl J Med"},{"key":"7571_CR55","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","volume":"384","author":"EJ Rubin","year":"2021","unstructured":"Rubin EJ, Retraction, Jayne DRW et al (2021) Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med 384:599\u2013609","journal-title":"N Engl J Med"},{"key":"7571_CR56","unstructured":"EMA (2026) EMA recommends revoking marketing authorisation for Tavneos | European Medicines Agency (EMA) [Internet] [cited 12 July 2026] Available from: https:\/\/www.ema.europa.eu\/en\/news\/ema-recommends-revoking-marketing-authorisation-tavneos"},{"key":"7571_CR57","unstructured":"FDA (2026) CDER proposes to withdraw approval of TAVNEOS. FDA [Internet] [cited 12 July 2026] Available from: https:\/\/www.fda.gov\/drugs\/drug-alerts-and-statements\/cder-proposes-withdraw-approval-tavneos"},{"key":"7571_CR58","doi-asserted-by":"publisher","first-page":"1921","DOI":"10.1056\/NEJMoa1702079","volume":"376","author":"ME Wechsler","year":"2017","unstructured":"Wechsler ME, Akuthota P, Jayne D et al (2017) Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med 376:1921\u20131932","journal-title":"N Engl J Med"},{"key":"7571_CR59","unstructured":"AstraZeneca (2025) Phase 3, Open-label Trial to Evaluate Safety, Pharmacokinetics, and Efficacy of Benralizumab in Children With Eosinophilic Diseases (CLIPS) [Internet]. clinicaltrials.gov [cited 28 Dec 2025] Available from: https:\/\/clinicaltrials.gov\/study\/NCT06512883"},{"key":"7571_CR60","unstructured":"Jianhua M (2025) A clinical study evaluating the safety and preliminary efficacy of universal allogeneic CAR T-cell therapy targeting CD19 and BCMA in patients with B cell mediated autoimmune disease [Internet]. clinicaltrials.gov [cited 28 Dec 2025] Available from: https:\/\/clinicaltrials.gov\/study\/NCT07305116"},{"key":"7571_CR61","unstructured":"Jianhua M (2024) Study of therapeutic efficacy of Anti-CD19 CAR-T cells in children with refractory ANCA-associated vasculitis [Internet]. clinicaltrials.gov [cited 28 Dec 2025] Available from: https:\/\/clinicaltrials.gov\/study\/NCT06508346"},{"key":"7571_CR62","unstructured":"Cartesian Therapeutics (2025) Descartes-08 for children, adolescents and young adults with childhood-onset systemic lupus erythematosus, ANCA-associated Vasculitis, Juvenile Myasthenia Gravis, and Juvenile Dermatomyositis [Internet]. clinicaltrials.gov [cited 28 Dec 2025] Available from: https:\/\/clinicaltrials.gov\/study\/NCT07089121"},{"key":"7571_CR63","unstructured":"Fate Therapeutics (2025) A phase 1 study of FT819 in B-cell mediated autoimmune disease [Internet]. clinicaltrials.gov [cited 28 Dec 2025] Available from: https:\/\/clinicaltrials.gov\/study\/NCT06308978"},{"key":"7571_CR64","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1007\/s004670050769","volume":"14","author":"N Besbas","year":"2000","unstructured":"Besbas N, Ozen S, Saatci U, Topaloglu R, Tinaztepe K, Bakkaloglu A (2000) Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children. Pediatr Nephrol 14:325\u2013327","journal-title":"Pediatr Nephrol"},{"key":"7571_CR65","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1007\/s004670000496","volume":"16","author":"SA Bakkaloglu","year":"2001","unstructured":"Bakkaloglu SA, Ekim M, T\u00fcmer N, Tulunay O, Ozer T (2001) Severe renal impairment in the case of classic polyarteritis nodosa. Pediatr Nephrol 16:148\u2013150","journal-title":"Pediatr Nephrol"},{"key":"7571_CR66","doi-asserted-by":"publisher","first-page":"2476","DOI":"10.1002\/art.38024","volume":"65","author":"D Eleftheriou","year":"2013","unstructured":"Eleftheriou D, Dillon MJ, Tullus K et al (2013) Systemic polyarteritis Nodosa in the Young: a single-center experience over thirty-two years. Arthritis Rheum 65:2476\u20132485","journal-title":"Arthritis Rheum"},{"key":"7571_CR67","doi-asserted-by":"publisher","first-page":"1061","DOI":"10.1002\/acr.24633","volume":"73","author":"SA Chung","year":"2021","unstructured":"Chung SA, Gorelik M, Langford CA et al (2021) 2021 American College of Rheumatology\/Vasculitis Foundation guideline for the management of polyarteritis nodosa. Arthritis Care Res 73:1061\u20131070","journal-title":"Arthritis Care Res"},{"key":"7571_CR68","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1016\/S0022-3476(05)80428-7","volume":"120","author":"S Ozen","year":"1992","unstructured":"Ozen S, Besbas N, Saatci U, Bakkaloglu A (1992) Diagnostic criteria for polyarteritis nodosa in childhood. J Pediatr 120:206\u2013209","journal-title":"J Pediatr"},{"key":"7571_CR69","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2021.102784","volume":"20","author":"E Conticini","year":"2021","unstructured":"Conticini E, Sota J, Falsetti P et al (2021) Biologic drugs in the treatment of polyarteritis nodosa and deficit of adenosine deaminase 2: a narrative review. Autoimmun Rev 20:102784","journal-title":"Autoimmun Rev"},{"key":"7571_CR70","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1007\/s11926-021-00983-2","volume":"23","author":"A Ho\u010devar","year":"2021","unstructured":"Ho\u010devar A, Tom\u0161i\u010d M, Perdan Pirkmajer K (2021) Clinical approach to diagnosis and therapy of polyarteritis nodosa. Curr Rheumatol Rep 23:14","journal-title":"Curr Rheumatol Rep"},{"key":"7571_CR71","first-page":"828","volume":"44","author":"ES Torun","year":"2025","unstructured":"Torun ES, \u00c7elen S (2025) Efficacy and safety of tocilizumab in polyarteritis nodosa and adenosine deaminase 2 deficiency: a systematic literature review. Clin Exp Rheumatol 44:828\u2013835","journal-title":"Clin Exp Rheumatol"},{"key":"7571_CR72","doi-asserted-by":"publisher","first-page":"2214","DOI":"10.1136\/annrheumdis-2016-209330","volume":"75","author":"D Rimar","year":"2016","unstructured":"Rimar D, Alpert A, Starosvetsky E et al (2016) Tofacitinib for polyarteritis nodosa: a tailored therapy. Ann Rheum Dis 75:2214\u20132216","journal-title":"Ann Rheum Dis"},{"key":"7571_CR73","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2021.635663","volume":"12","author":"R Bursi","year":"2021","unstructured":"Bursi R, Cafaro G, Perricone C et al (2021) Contribution of Janus-kinase\/signal transduction activator of transcription pathway in the pathogenesis of vasculitis: a possible treatment target in the upcoming future. Front Pharmacol 12:635663","journal-title":"Front Pharmacol"},{"key":"7571_CR74","first-page":"52","volume":"37","author":"HE S\u00f6nmez","year":"2019","unstructured":"S\u00f6nmez HE, Arma\u011fan B, Ayan G et al (2019) Polyarteritis nodosa: lessons from 25 years of experience. Clin Exp Rheumatol 37:52\u201356. Suppl 117","journal-title":"Clin Exp Rheumatol"},{"key":"7571_CR75","doi-asserted-by":"publisher","first-page":"2565","DOI":"10.3390\/diagnostics12102565","volume":"12","author":"DP Misra","year":"2022","unstructured":"Misra DP, Jain N, Ora M, Singh K, Agarwal V, Sharma A (2022) Outcome measures and biomarkers for disease assessment in Takayasu arteritis. Diagnostics 12:2565","journal-title":"Diagnostics"},{"key":"7571_CR76","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2024.1495644","volume":"11","author":"R Ni","year":"2024","unstructured":"Ni R, Kohler MJ (2024) What is new in imaging to assist in the diagnosis of giant cell arteritis and Takayasu\u2019s arteritis since the EULAR and ACR\/VF recommendations? Front Med 11:1495644","journal-title":"Front Med"},{"key":"7571_CR77","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1093\/rheumatology\/key040","volume":"58","author":"E Tombetti","year":"2019","unstructured":"Tombetti E, Mason JC (2019) Takayasu arteritis: advanced understanding is leading to new horizons. Rheumatology 58:206\u2013219","journal-title":"Rheumatology"},{"key":"7571_CR78","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2021.814075","volume":"8","author":"F Regola","year":"2022","unstructured":"Regola F, Uzzo M, Toniati P, Trezzi B, Sinico RA, Franceschini F (2022) Novel therapies in Takayasu arteritis. Front Med 8: 814075","journal-title":"Front Med"},{"key":"7571_CR79","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1136\/annrheumdis-2019-215672","volume":"79","author":"B Hellmich","year":"2020","unstructured":"Hellmich B, Agueda A, Monti S et al (2020) 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 79:19\u201330","journal-title":"Ann Rheum Dis"},{"key":"7571_CR80","doi-asserted-by":"publisher","first-page":"2427","DOI":"10.1093\/rheumatology\/kez630","volume":"59","author":"Y Nakaoka","year":"2020","unstructured":"Nakaoka Y, Isobe M, Tanaka Y et al (2020) Long-term efficacy and safety of tocilizumab in refractory Takayasu arteritis: final results of the randomized controlled phase 3 TAKT study. Rheumatology 59:2427\u20132434","journal-title":"Rheumatology"},{"key":"7571_CR81","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103275","volume":"22","author":"DP Misra","year":"2023","unstructured":"Misra DP, Singh K, Rathore U et al (2023) The effectiveness of tocilizumab and its comparison with tumor necrosis factor alpha inhibitors for Takayasu Arteritis: a systematic review and meta-analysis. Autoimmun Rev 22:103275","journal-title":"Autoimmun Rev"},{"key":"7571_CR82","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1080\/03009742.2017.1278788","volume":"47","author":"R Yachoui","year":"2018","unstructured":"Yachoui R, Kreidy M, Siorek M, Sehgal R (2018) Successful treatment with ustekinumab for corticosteroid- and immunosuppressant-resistant Takayasu\u2019s arteritis. Scand J Rheumatol 47:246\u2013247","journal-title":"Scand J Rheumatol"},{"key":"7571_CR83","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1002\/art.40037","volume":"69","author":"CA Langford","year":"2017","unstructured":"Langford CA, Cuthbertson D, Ytterberg SR et al (2017) A randomized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of Takayasu arteritis. Arthritis Rheumatol 69:846\u2013853","journal-title":"Arthritis Rheumatol"},{"key":"7571_CR84","doi-asserted-by":"publisher","first-page":"1989","DOI":"10.1007\/s00296-019-04390-w","volume":"39","author":"T Mutoh","year":"2019","unstructured":"Mutoh T, Ishii T, Shirai T et al (2019) Refractory Takayasu arteritis successfully treated with rituximab: case-based review. Rheumatol Int 39:1989\u20131994","journal-title":"Rheumatol Int"},{"key":"7571_CR85","doi-asserted-by":"publisher","first-page":"rkaa082","DOI":"10.1093\/rap\/rkaa082","volume":"5","author":"S-J Saur","year":"2021","unstructured":"Saur S-J, Horger M, Henes J (2021) Successful treatment with the IL12\/IL23 antagonist ustekinumab in a patient with refractory Takayasu arteritis. Rheumatol Adv Pract 5:rkaa082","journal-title":"Rheumatol Adv Pract"},{"key":"7571_CR86","unstructured":"Dang A (2016) China Takayasu Arteritis Registry (CTA Registry) [Internet]. clinicaltrials.gov [cited 4 Jan 2026] Available from: https:\/\/clinicaltrials.gov\/study\/NCT02964364"},{"key":"7571_CR87","unstructured":"China National Center for Cardiovascular Diseases (2019) Registration Study of Takayasu\u2019s Arteritis in China [Internet]. clinicaltrials.gov [cited 4 Jan 2026] Available from: https:\/\/clinicaltrials.gov\/study\/NCT03199183"},{"key":"7571_CR88","doi-asserted-by":"publisher","DOI":"10.1093\/rap\/rkaf013","volume":"9","author":"H Yoshifuji","year":"2025","unstructured":"Yoshifuji H, Ishii T, Ohashi H et al (2025) Phase 3, multicentre, randomized, double-blind, placebo-controlled, parallel-group study of ustekinumab in patients with Takayasu arteritis. Rheumatol Adv Pract 9:rkaf013","journal-title":"Rheumatol Adv Pract"},{"key":"7571_CR89","doi-asserted-by":"publisher","first-page":"995","DOI":"10.1007\/s00431-017-2937-5","volume":"176","author":"SM Dietz","year":"2017","unstructured":"Dietz SM, van Stijn D, Burgner D et al (2017) Dissecting Kawasaki disease: a state-of-the-art review. Eur J Pediatr 176:995\u20131009","journal-title":"Eur J Pediatr"},{"key":"7571_CR90","doi-asserted-by":"publisher","first-page":"e481","DOI":"10.1161\/CIR.0000000000001295","volume":"150","author":"P-N Jone","year":"2024","unstructured":"Jone P-N, Tremoulet A, Choueiter N et al (2024) Update on diagnosis and management of Kawasaki disease: a scientific statement from the American Heart Association. Circulation 150:e481\u2013e500","journal-title":"Circulation"},{"key":"7571_CR91","doi-asserted-by":"publisher","first-page":"1135","DOI":"10.1111\/ped.14326","volume":"62","author":"T Kobayashi","year":"2020","unstructured":"Kobayashi T, Ayusawa M, Suzuki H et al (2020) Revision of diagnostic guidelines for Kawasaki disease (6th revised edition). Pediatr Int 62:1135\u20131138","journal-title":"Pediatr Int"},{"key":"7571_CR92","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/S2352-4642(21)00270-4","volume":"5","author":"JC Burns","year":"2021","unstructured":"Burns JC, Roberts SC, Tremoulet AH et al (2021) Infliximab versus second intravenous immunoglobulin for treatment of resistant Kawasaki disease in the USA (KIDCARE): a randomised, multicentre comparative effectiveness trial. Lancet Child Adolesc Health 5:852\u2013861","journal-title":"Lancet Child Adolesc Health"},{"key":"7571_CR93","doi-asserted-by":"publisher","first-page":"1128","DOI":"10.1016\/S0140-6736(18)32003-8","volume":"393","author":"H Hamada","year":"2019","unstructured":"Hamada H, Suzuki H, Onouchi Y, Ebata R, Terai M, Fuse S, Okajima Y, Kurotobi S, Hirai K, Soga T, Ishiguchi Y, Okuma Y, Takada N, Yanai M, Sato J, Nakayashiro M, Ayusawa M, Yamamoto E, Nomura Y, Hashimura Y, Ouchi K, Masuda H, Takatsuki S, Hirono K, Ariga T, Higaki T, Otsuki A, Terauchi M, Aoyagi R, Sato T, Fujii Y, Fujiwara T, Hanaoka H, Hata A, KAICA trial Investigators (2019) Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial. Lancet 393:1128\u20131137. https:\/\/doi.org\/10.1016\/S0140-6736(18)32003-8","journal-title":"Lancet"},{"key":"7571_CR94","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2025.114479","volume":"279","author":"I Bellicini","year":"2025","unstructured":"Bellicini I, Bainto E, Shimizu C, Burns JC, Tremoulet AH (2025) Cyclosporine treatment in patients with Kawasaki disease and coronary artery aneurysms or treatment resistance. J Pediatr 279: 114479","journal-title":"J Pediatr"},{"key":"7571_CR95","doi-asserted-by":"publisher","first-page":"647","DOI":"10.1056\/NEJMoa2510198","volume":"394","author":"R Lafayette","year":"2026","unstructured":"Lafayette R, Barbour SJ, Brenner RM et al (2026) A phase 3 trial of atacicept in patients with IgA nephropathy. N Engl J Med 394:647\u2013657","journal-title":"N Engl J Med"},{"key":"7571_CR96","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1056\/NEJMoa2311155","volume":"390","author":"ME Wechsler","year":"2024","unstructured":"Wechsler ME, Nair P, Terrier B, Walz B, Bourdin A, Jayne DRW, Jackson DJ, Roufosse F, B\u00f6rjesson Sj\u00f6 L, Fan Y, Jison M, McCrae C, Necander S, Shavit A, Walton C, Merkel PA, MANDARA Study Group (2024) Benralizumab versus mepolizumab for eosinophilic granulomatosis with polyangiitis. N Engl J Med 390:911\u2013921","journal-title":"N Engl J Med"}],"container-title":["Pediatric Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-026-07571-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00467-026-07571-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00467-026-07571-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T20:03:17Z","timestamp":1792440197000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00467-026-07571-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,19]]},"references-count":96,"alternative-id":["7571"],"URL":"https:\/\/doi.org\/10.1007\/s00467-026-07571-5","relation":{},"ISSN":["0931-041X","1432-198X"],"issn-type":[{"value":"0931-041X","type":"print"},{"value":"1432-198X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,19]]},"assertion":[{"value":"13 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"31 July 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 September 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 October 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable. This review did not involve studies with human participants or animals performed by any of the authors.","order":2,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}}]}}