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Compared to adults, pediatric IgA nephropathy has a more acute presentation, most frequently with synpharyngitic macrohematuria and histologically with more intense inflammation and less intense chronic damage. Management of these conditions is controversial and supported by little high-quality evidence. The paucity of evidence is due to the disease heterogeneity, its inter-ethnic variability, and the difficulty of extrapolating data from adult studies due to the peculiarities of the condition in children. IgA vasculitis with nephritis is a kidney manifestation of a systemic disorder, typical of the pediatric age, in which both the diagnosis of kidney involvement and its management are poorly defined, and an interdisciplinary approach is crucial. Both conditions can have a profound and long-lasting impact on kidney function and the global health of affected children. The International Pediatric Nephrology Association has therefore convened a diverse international group of experts from different disciplines to provide guidance on the recommended management of these conditions in children and to establish common definitions and define priorities for future high-quality, evidence-based collaborative studies for the benefit of children.<\/jats:p>","DOI":"10.1007\/s00467-024-06502-6","type":"journal-article","created":{"date-parts":[[2024,9,27]],"date-time":"2024-09-27T09:02:59Z","timestamp":1727427779000},"page":"533-569","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":113,"title":["IPNA clinical practice recommendations for the diagnosis and management of children with IgA nephropathy and IgA vasculitis nephritis"],"prefix":"10.1007","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2448-1959","authenticated-orcid":false,"given":"Marina","family":"Vivarelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Samuel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosanna","family":"Coppo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Barratt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melvin","family":"Bonilla-Felix","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dieter","family":"Haffner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisha","family":"Gibson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Haas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maher Ahmed","family":"Abdel-Hafez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Adragna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Brogan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siah","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isaac","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi-Hong","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mukta","family":"Mantan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Shima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Shimuzu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qian","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hernan","family":"Trimarchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre","family":"Hahn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Hodson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken","family":"Pfister","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Areefa","family":"Alladin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivia","family":"Boyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Nakanishi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the International Pediatric Nephrology Association","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,9,27]]},"reference":[{"key":"6502_CR1","doi-asserted-by":"publisher","first-page":"2402","DOI":"10.1056\/NEJMra1206793","volume":"368","author":"RJ Wyatt","year":"2013","unstructured":"Wyatt RJ, Julian BA (2013) IgA Nephropathy. 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These did not influence the content of this guideline.SS has no conflict of interest to declare.RC has no conflict of interest to declare.JB reports research grants from Argenx, Calliditas Therapeutics, Chinook Therapeutics, Galapagos, GlaxoSmithKline, Novartis, and Travere Therapeutics and is medical and\/or scientific advisor to Alnylam Pharmaceuticals, Argenx, Astellas Pharma, BioCryst Pharmaceuticals, Calliditas Therapeutics, Chinook Therapeutics, Dimerix, Galapagos, Novartis, Omeros, Travere Therapeutics, UCB, Vera Therapeutics, and Visterra.MBF holds Advisor-Speaker contracts with Natera and Sanofi Pharmaceutical.DH has no conflicts of interest to declare.KG reports consultative roles with Travere Inc. and research support from Travere Inc., Roche Inc., and Aurinia Inc.MH has received consulting fees from Argenx, AstraZeneca, CareDx, Novartis, Travere, and Vera Therapeutics.MAAH has no conflict of interest to disclose.MA is a member of advisory boards and participates\u00a0in scientific events receiving\u00a0honoraria from Alexion and Gador. This did not influence the content of this guideline.PB has no conflict of interest to declare.SK has no conflict of interest to declare.ILD has no conflict of interest to declare.ZHL has no conflict of interest to declare.MM has no conflict of interest to declare.YS has no conflict of interest to disclose.MS has no conflict of interest to declare.QS has no conflict of interest to declare.HT has no conflict of interest to declare.DH has no conflict of interest to declare.EH has no conflict of interest to declare.KP has no conflict of interest to declare.AA has no conflict of interest to declare.OB has participated in sponsored trials and has received speaker\/consultant fees from Alexion, Alnylam, Biocodex, Novartis, PureSpring, Travere, and Vifor. These did not influence the content of this guideline.KN\u00a0has participated in sponsored trials and has received speaker\/consultant fees from\u00a0AstraZeneca,\u00a0Alexion,\u00a0KyowaKirin,\u00a0Novartis,\u00a0Sumitomo Pharma, and Chugai Pharmaceutical. These did not influence the content of this guideline.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}