Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)

Marek Bohm, Maria I. Gonzalez Fernandez, Seza Ozen, Angela Pistorio, Pavla Dolezalova, Paul Brogan, Giancarlo Barbano, Claudia Sengler, Marisa Klein-Gitelman, Pierre Quartier, Anders Fasth, Troels Herlin, Maria Teresa R.A. Terreri, Susan Nielsen, Marion A.J. van Rossum, Tadej Avcin, Esteban R. Castell, Ivan Foeldvari, Dirk Foell, Anuela KondiIsabelle Koné-Paut, Rolf Michael Kuester, Hartmut Michels, Nico Wulffraat, Halima B. Amer, Clara Malattia, Alberto Martini, Nicolino Ruperto*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

55 Scopus citations


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.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.

Original languageEnglish (US)
Article number18
JournalPediatric Rheumatology
Issue number1
StatePublished - May 26 2014


  • Clinical picture of disease
  • Clinical study
  • Comparison with literature
  • Granulomatosis with polyangiitis
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Rheumatology

Fingerprint Dive into the research topics of 'Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)'. Together they form a unique fingerprint.

Cite this