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dc.contributor.authorBohm, Marek
dc.contributor.authorGonzalez Fernandez, Maria Isabel
dc.contributor.authorOzen, Seza
dc.contributor.authorPistorio, Angela
dc.contributor.authorDolezalova, Pavla
dc.contributor.authorBrogan, Paul
dc.contributor.authorBarbano, Giancarlo
dc.contributor.authorSengler, Claudia
dc.contributor.authorKlein-Gitelman, Marisa
dc.contributor.authorQuartier, Pierre
dc.contributor.authorFasth, Anders
dc.contributor.authorHerlin, Troels
dc.contributor.authorTerreri, Maria Teresa R A
dc.contributor.authorNielsen, Susan
dc.contributor.authorvan Rossum, Marion A J
dc.contributor.authorAvcin, Tadej
dc.contributor.authorCastell, Esteban Rodolfo
dc.contributor.authorFoeldvari, Ivan
dc.contributor.authorFoell, Dirk
dc.contributor.authorKondi, Anuela
dc.contributor.authorKoné-Paut, Isabelle
dc.contributor.authorKuester, Rolf-Michael
dc.contributor.authorMichels, Hartmut
dc.contributor.authorWulffraat, Nico
dc.contributor.authorAmer, Halima Ben
dc.contributor.authorMalattia, Clara
dc.contributor.authorMartini, Alberto
dc.contributor.authorRuperto, Nicolino
dc.date.accessioned2019-12-10T10:35:15Z
dc.date.available2019-12-10T10:35:15Z
dc.date.issued2014
dc.identifier.issn1546-0096
dc.identifier.urihttps://doi.org/10.1186/1546-0096-12-18
dc.identifier.urihttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4041043/
dc.identifier.urihttp://hdl.handle.net/11655/13851
dc.description.abstractBackground Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients., Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature. Findings The 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series. Conclusions Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.
dc.relation.isversionof10.1186/1546-0096-12-18
dc.rightsinfo:eu-repo/semantics/openAccess
dc.titleClinical Features Of Childhood Granulomatosis With Polyangiitis (Wegener’S Granulomatosis)
dc.typeinfo:eu-repo/semantics/article
dc.typeinfo:eu-repo/semantics/publishedVersion
dc.relation.journalPediatric Rheumatology Online Journal
dc.contributor.departmentÇocuk Sağlığı ve Hastalıkları
dc.identifier.volume12
dc.identifier.startpage18
dc.description.indexPubMed
dc.description.indexWoS
dc.description.indexScopus


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