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

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.
AuthorsMarek Bohm, Maria Isabel 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 Rodolfo Castell, Ivan Foeldvari, Dirk Foell, Anuela Kondi, Isabelle Koné-Paut, Rolf-Michael Kuester, Hartmut Michels, Nico Wulffraat, Halima Ben Amer, Clara Malattia, Alberto Martini, Nicolino Ruperto
JournalPediatric rheumatology online journal (Pediatr Rheumatol Online J) Vol. 12 Pg. 18 ( 2014) ISSN: 1546-0096 [Print] England
PMID24891844 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Antibodies, Antineutrophil Cytoplasmic
Topics
  • Adolescent
  • Adult
  • Age Factors
  • Age of Onset
  • Antibodies, Antineutrophil Cytoplasmic (blood)
  • Biopsy
  • Child
  • Child, Preschool
  • Female
  • Granuloma, Respiratory Tract (immunology, pathology)
  • Granulomatosis with Polyangiitis (diagnosis, epidemiology, immunology, physiopathology)
  • Humans
  • International Cooperation
  • Male
  • Organ Specificity (immunology)
  • Prognosis

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