title: Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis).
abstract: BACKGROUND
  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.
  
  
  RESULTS
  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.
authors.affiliation: Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy ; Charles University in Prague, 1st Medical Faculty and General University Hospital in Prague, Prague, Czech Republic.
authors.forename: Marek
authors.initials: M
authors.lastname: Bohm
authors.affiliation: Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy.
authors.forename: Maria Isabel
authors.initials: MI
authors.lastname: Gonzalez Fernandez
authors.affiliation: Department of Pediatric Rheumatology and Nephrology, Hacettepe University Children's Hospital, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.affiliation: Istituto Giannina Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Pistorio
authors.affiliation: Charles University in Prague, 1st Medical Faculty and General University Hospital in Prague, Prague, Czech Republic.
authors.forename: Pavla
authors.initials: P
authors.lastname: Dolezalova
authors.affiliation: Department of Rheumatology, Great Ormond St Hospital, NHS Foundation Trust, London, UK.
authors.forename: Paul
authors.initials: P
authors.lastname: Brogan
authors.affiliation: Istituto Giannina Gaslini, Nefrologia, Genova, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Barbano
authors.affiliation: Department of Pediatrics, Division of Pneumology and Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Claudia
authors.initials: C
authors.lastname: Sengler
authors.affiliation: Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
authors.forename: Marisa
authors.initials: M
authors.lastname: Klein-Gitelman
authors.affiliation: Université Paris-Descartes, Institut IMAGINE, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pediatrique, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Quartier
authors.affiliation: Department of Pediatrics, The Queen Silvia Children's Hospital, University of Gothenburg, Göteborg, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Fasth
authors.affiliation: Department of Pediatrics, Skejby Sygehus, Aarhus University Hospital, Pediatric Rheumatology Clinic, Aarhus, Denmark.
authors.forename: Troels
authors.initials: T
authors.lastname: Herlin
authors.affiliation: Universidade Federal de São Paulo, Pediatrics, São Paulo, Brazil.
authors.forename: Maria Teresa R A
authors.initials: MT
authors.lastname: Terreri
authors.affiliation: Juliane Marie Centret, Rigshospitalet, Pediatrisk klinik II, Copenhagen, Denmark.
authors.forename: Susan
authors.initials: S
authors.lastname: Nielsen
authors.affiliation: Department of Pediatrics, Emma Children Hospital AMC, Amsterdam, Netherlands.
authors.forename: Marion A J
authors.initials: MA
authors.lastname: van Rossum
authors.affiliation: Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
authors.forename: Tadej
authors.initials: T
authors.lastname: Avcin
authors.affiliation: Hospital Dr Felipe Glasman, Rheumatolgy Section, Bahía Blanca, Buenos Aires, Argentina.
authors.forename: Esteban Rodolfo
authors.initials: ER
authors.lastname: Castell
authors.affiliation: Hamburger Zentrum für Kinder- und Jugendrheumatologie, Klinikum Eilbek Hs.6, Hamburg, Germany.
authors.forename: Ivan
authors.initials: I
authors.lastname: Foeldvari
authors.affiliation: Department of Pediatric Rheumatology and Immunology, University Children's Hospital, Muenster, Germany.
authors.forename: Dirk
authors.initials: D
authors.lastname: Foell
authors.affiliation: Pediatric Department, University Hospital Centre, Tirana, Albania.
authors.forename: Anuela
authors.initials: A
authors.lastname: Kondi
authors.affiliation: CHU Le Kremlin Bicêtre, APHP, University of Paris Sud, CEREMAI (Centre de référence national des maladies auto-inflammatoires, rhumatologie pédiatrique), Le Kremlin Bicêtre, Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
authors.affiliation: Asklepios Klinik Altona, Hamburg, Germany.
authors.forename: Rolf-Michael
authors.initials: RM
authors.lastname: Kuester
authors.affiliation: Kinderklinik Garmisch-Partenkirchen gGmbH, Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany.
authors.forename: Hartmut
authors.initials: H
authors.lastname: Michels
authors.affiliation: Department of Pediatric Immunology and Rheumatology, Wilhelmina Kinderziekenhuis, Utrecht, Netherlands.
authors.forename: Nico
authors.initials: N
authors.lastname: Wulffraat
authors.affiliation: Benghazi Children Hospital - Benghazi, MUB - Rheumatology Clinic, Benghazi, Lybia.
authors.forename: Halima Ben
authors.initials: HB
authors.lastname: Amer
authors.affiliation: Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy.
authors.forename: Clara
authors.initials: C
authors.lastname: Malattia
authors.affiliation: Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy ; Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.affiliation: Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy.
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D015769:Granuloma, Respiratory Tract / Q000276:immunology / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007391:International Cooperation; D008297:Male; D009928:Organ Specificity / Q000276:immunology; D011379:Prognosis
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Clinical picture of disease; Clinical study; Comparison with literature; Granulomatosis with polyangiitis; Wegener’s granulomatosis