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

Pediatr Rheumatol Online J. 2014 May 26;12:18. doi: 10.1186/1546-0096-12-18. eCollection 2014.

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.

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.

Keywords: Clinical picture of disease; Clinical study; Comparison with literature; Granulomatosis with polyangiitis; Wegener’s granulomatosis.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Age of Onset
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Biopsy
  • Child
  • Child, Preschool
  • Female
  • Granuloma, Respiratory Tract* / immunology
  • Granuloma, Respiratory Tract* / pathology
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / epidemiology
  • Granulomatosis with Polyangiitis* / immunology
  • Granulomatosis with Polyangiitis* / physiopathology
  • Humans
  • International Cooperation
  • Male
  • Organ Specificity / immunology
  • Prognosis

Substances

  • Antibodies, Antineutrophil Cytoplasmic