{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T09:08:15Z","timestamp":1785229695106,"version":"3.55.0"},"reference-count":40,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,3,18]],"date-time":"2025-03-18T00:00:00Z","timestamp":1742256000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Wegener\u2019s granulomatosis (WG), or granulomatosis with polyangiitis (GPA), is a rare autoimmune disease that can cause inflammation in various organs, including the kidneys. Renal involvement in GPA is a major cause of morbidity and mortality in both adults and children, and early detection and effective treatment are essential for preventing renal failure. This review aims to summarize the current evidence on the incidence, clinical features, treatment, and outcomes of renal involvement in children with Wegener\u2019s granulomatosis. The incidence of renal involvement in children with GPA ranged from 26% to 56%. Renal involvement is a common and serious complication of GPA in children, and early detection and effective treatment are crucial for preventing renal failure. The most common clinical features were proteinuria, hematuria, and reduced glomerular filtration rate. The majority of children with renal involvement in GPA required treatment with corticosteroids and immunosuppressive agents. The treatment outcomes varied among the studies, with some children achieving remission of renal involvement while others developed end-stage renal disease. Although most features are the same in children and adult patients, this review summed up some important differences between these two different populations. Further studies are needed to identify the most effective treatment strategies for renal involvement in children with GPA.<\/jats:p>","DOI":"10.3390\/medicina61030534","type":"journal-article","created":{"date-parts":[[2025,3,18]],"date-time":"2025-03-18T12:03:06Z","timestamp":1742299386000},"page":"534","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Granulomatosis with Polyangiitis: A Focus on Differences and Similarities Between Child and Adult Patients"],"prefix":"10.3390","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6333-5009","authenticated-orcid":false,"given":"Vincenzo","family":"Calabrese","sequence":"first","affiliation":[{"name":"Department of Medicine and Surgery, University of Enna \u201cKore\u201d, 94100 Enna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romina","family":"Gallizzi","sequence":"additional","affiliation":[{"name":"Unit of Pediatric Nephrology and Rheumatology, University of Messina, 98125 Messina, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-8676-2013","authenticated-orcid":false,"given":"Alessandra","family":"Spagnolo","sequence":"additional","affiliation":[{"name":"Unit of Pediatric Nephrology and Rheumatology, University of Messina, 98125 Messina, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariateresa","family":"Zicarelli","sequence":"additional","affiliation":[{"name":"Unit of Nephrology and Dialysis, Department of Clinical and Experimental Medicine, A.O.U. \u201cG. Martino\u201d, University of Messina, 98125 Messina, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Sutera","sequence":"additional","affiliation":[{"name":"Unit of Pediatric Nephrology and Rheumatology, University of Messina, 98125 Messina, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Farina","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences, University of Catanzaro \u201cMagna Graecia\u201d, 88100 Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4396-5032","authenticated-orcid":false,"given":"Valeria","family":"Cernaro","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences, University of Catanzaro \u201cMagna Graecia\u201d, 88100 Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4279-6559","authenticated-orcid":false,"given":"Domenico","family":"Santoro","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Sciences, University of Catanzaro \u201cMagna Graecia\u201d, 88100 Catanzaro, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,3,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3413","DOI":"10.1002\/art.24876","article-title":"Classification, presentation, and initial treatment of Wegener\u2019s granulomatosis in childhood","volume":"60","author":"Cabral","year":"2009","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1007\/s00431-004-1464-3","article-title":"Wegener granulomatosis in childhood and adolescence","volume":"163","author":"Frosch","year":"2004","journal-title":"Eur. J. Pediatr."},{"key":"ref_3","first-page":"221","article-title":"Wegener granulomatosis","volume":"41","author":"Westman","year":"2015","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1007\/s00467-002-0914-2","article-title":"Clinical features in 17 paediatric patients with Wegener granulomatosis","volume":"17","author":"Belostotsky","year":"2002","journal-title":"Pediatr. Nephrol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S1521-6616(02)00026-8","article-title":"Diagnostic and pathogenetic role of antineutrophil cytoplasmic autoantibodies [review]","volume":"106","year":"2003","journal-title":"Clin. Immunol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1002\/art.22774","article-title":"Clinical features and outcome of pediatric Wegener\u2019s granulomatosis","volume":"57","author":"Akikusa","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"488","DOI":"10.7326\/0003-4819-116-6-488","article-title":"Wegener granulomatosis: An analysis of 158 patients","volume":"116","author":"Hoffman","year":"1992","journal-title":"Ann. Intern. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/S0022-3476(05)83482-1","article-title":"Wegener granulomatosis in children and Pediatric Wegener\u2019s Granulomatosis 843 adolescents: Clinical presentation and outcome","volume":"122","author":"Rottem","year":"1993","journal-title":"J. Pediatr."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1177\/019459988609400320","article-title":"Presentation of Wegener\u2019s granulomatosis in young patients","volume":"94","author":"Halstead","year":"1986","journal-title":"Otolaryngol. Head Neck Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1007\/s004670050043","article-title":"Wegener granulomatosis in children and young adults: A case study of ten patients","volume":"14","author":"Stegmayr","year":"2000","journal-title":"Pediatr. Nephrol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/S0022-3476(85)80346-2","article-title":"Wegener granulomatosis in pediatric patients","volume":"106","author":"Hall","year":"1985","journal-title":"J. Pediatr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1111\/j.1526-0968.2001.00300.x","article-title":"Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis","volume":"5","author":"Gaskin","year":"2001","journal-title":"Ther. Apher."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1056\/NEJMoa0909905","article-title":"Rituximab versus cyclophosphamide for ANCA-associated vasculitis","volume":"363","author":"Stone","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Potentas-Policewicz, M., and Fijolek, J. (2024). Granulomatosis with polyangiitis: Clinical characteristics and updates in diagnosis. Front. Med., 11.","DOI":"10.3389\/fmed.2024.1369233"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"NP17","DOI":"10.1177\/11206721241295298","article-title":"Avacopan in the treatment of refractory scleritis secondary to granulomatosis with polyangiitis: A case report","volume":"35","author":"Bober","year":"2025","journal-title":"Eur. J. Ophthalmol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e236236","DOI":"10.1136\/bcr-2020-236236","article-title":"Long-term use and remission of granulomatosis with polyangiitis with the oral C5a receptor inhibitor avacopan","volume":"13","author":"Ennis","year":"2020","journal-title":"BMJ Case Rep."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1550","DOI":"10.1002\/acr.24590","article-title":"Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody-Associated Vasculitis","volume":"74","author":"Morishita","year":"2022","journal-title":"Arthritis Care Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1136\/annrheumdis-2019-eular.8509","article-title":"SP0182 treatment of AAV","volume":"78","author":"Jayne","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_19","first-page":"w14103","article-title":"The role of rituximab in the treatment of ANCA-associated vasculitides (AAV)","volume":"145","author":"Daikeler","year":"2015","journal-title":"Swiss Med. Wkly."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"3151","DOI":"10.1002\/art.38788","article-title":"Rituximab in ANCA-Associated Vasculitis-Immune Tolerance Network Research Group. Rituximab for the treatment of relapses in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"66","author":"Miloslavsky","year":"2014","journal-title":"Arthritis Rheumatol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s00467-016-3559-2","article-title":"Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: An update on renal management","volume":"33","author":"Oni","year":"2018","journal-title":"Pediatr. Nephrol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1911","DOI":"10.1007\/s00467-012-2198-5","article-title":"ANCA-associated glomerulonephritis\/systemic vasculitis in childhood: Clinical features\u2013outcome","volume":"27","author":"Siomou","year":"2012","journal-title":"Pediatr. Nephrol."},{"key":"ref_23","first-page":"71","article-title":"Granulomatosis de Wegener. Reporte de un caso pedi\u00e1trico y revisi\u00f3n de la literatura [Wegener\u2019s granulomatosis. Report of a pediatric case and review of the literature]","volume":"50","year":"2003","journal-title":"Rev. Alerg. Mex."},{"key":"ref_24","first-page":"301","article-title":"K\u0142ebuszkowe choroby nerek jako przyczyna schy\u0142kowej niewydolno\u015bci nerek [Glomerulopathies as causes of end-stage renal disease in children]","volume":"26","author":"Adamczuk","year":"2009","journal-title":"Pol. Merkur. Lekarski."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1002\/art.1780330804","article-title":"Members and Consultants of the American College of Rheumatology Subcommittee on Classification of Vasculitis. Illustrated histopathologic classification criteria for selected vasculitis syndromes","volume":"33","author":"Lie","year":"1990","journal-title":"Arthritis Rheum."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1093\/ndt\/16.5.953","article-title":"Renal histopathology and clinical course in 94 patients with Wegener\u2019s granulomatosis","volume":"16","author":"Bostad","year":"2001","journal-title":"Nephrol. Dial. Transpl."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1007\/s00467-003-1365-0","article-title":"A boy with consecutive development of SLE and Wegener granulomatosis","volume":"19","author":"Oner","year":"2004","journal-title":"Pediatr. Nephrol."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Makovetskaya, G., Mazur, L., and Balashova, E. (2019). Granulomatous Interstitial Nephritis in Children Resulting from Wegener\u2019s Granulomatosis, Crohn\u2019s Disease, or Sarcoidosis. Sarcoidosis and Granulomatosis-Diagnosis and Management, IntechOpen.","DOI":"10.5772\/intechopen.84491"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"440","DOI":"10.4065\/72.5.440","article-title":"Vitamin D metabolite-mediated hypercalcemia in Wegener\u2019s granulomatosis","volume":"72","author":"Bosch","year":"1997","journal-title":"Mayo Clin. Proc."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1159\/000168633","article-title":"Hypercalcemia associated with Wegener\u2019s granulomatosis and hyperparathyroidism: Etiology and management","volume":"13","author":"Edelson","year":"1993","journal-title":"Am. J. Nephrol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1097\/00000441-199408000-00011","article-title":"Case report: Hypercalcemia with inappropriate 1,25-dihydroxyvitamin D in Wegener\u2019s granulomatosis","volume":"308","author":"Shaker","year":"1994","journal-title":"Am. J. Med. Sci."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/S0022-3476(98)70453-6","article-title":"Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: A single-center experience","volume":"132","author":"Valentini","year":"1998","journal-title":"J. Pediatr."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1093\/ndt\/15.5.611","article-title":"Wegener\u2019s granulomatosis: Clinical course in 108 patients with renal involvement","volume":"15","author":"Iversen","year":"2000","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/S0022-2143(97)90157-8","article-title":"Wegener\u2019s granulomatosis: A meta-analysis of 349 literary case reports","volume":"129","author":"Bajema","year":"1997","journal-title":"J. Lab. Clin. Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1681\/ASN.V7123","article-title":"Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis","volume":"7","author":"Hogan","year":"1996","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_36","first-page":"1237","article-title":"Longterm outcome of Wegener\u2019s granulomatosis in patients with renal disease requiring dialysis","volume":"27","author":"Mekhail","year":"2000","journal-title":"J. Rheumatol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1007\/s10238-021-00762-4","article-title":"Clinical features and outcomes of anti-neutrophil cytoplasmic autoantibody-associated vasculitis in Chinese childhood-onset patients","volume":"22","author":"Meng","year":"2022","journal-title":"Clin. Exp. Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"2514","DOI":"10.1002\/art.39729","article-title":"Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener\u2019s): An ARChiVe Cohort Study","volume":"68","author":"Cabral","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1111\/j.1525-1470.1998.tb01363.x","article-title":"Wegener\u2019s granulomatosis: Case report and literature review","volume":"15","author":"Stein","year":"1998","journal-title":"Pediatr. Dermatol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1002\/lary.26013","article-title":"The presentation and management of granulomatosis with polyangiitis (Wegener\u2019s Granulomatosis) in the pediatric airway","volume":"127","author":"Lee","year":"2017","journal-title":"Laryngoscope"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/61\/3\/534\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T16:56:12Z","timestamp":1760028972000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/61\/3\/534"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,18]]},"references-count":40,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,3]]}},"alternative-id":["medicina61030534"],"URL":"https:\/\/doi.org\/10.3390\/medicina61030534","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,18]]}}}