Wegener granulomatosis in children and adolescents: clinical presentation and outcome

J Pediatr. 1993 Jan;122(1):26-31. doi: 10.1016/s0022-3476(05)83482-1.


We prospectively studied and compared clinical features, treatment, course of illness, and long-term morbidity and mortality rates for Wegener granulomatosis in 23 childhood-onset patients with those of 135 adult-onset patients who were studied concurrently. Treatment was usually provided with glucocorticoids and cyclophosphamide. The mean follow-up period was 8.7 years for childhood-onset and 7.6 years for adult-onset Wegener granulomatosis. Most aspects of Wegener granulomatosis were similar in childhood-onset and adult-onset patients. Permanent morbidity from disease occurred in 86% of both groups. However, some features were significantly different. Wegener granulomatosis in childhood-onset patients was complicated five times more often by subglottic stenosis and twice as often by nasal deformity. Treatment-related permanent morbidity occurred in 22% of childhood-onset patients and 45% of adult-onset patients. After similar periods of cyclophosphamide therapy and follow-up, cyclophosphamide-related malignancies were less likely (0% vs 11%) to have developed in childhood-onset patients. Although 89% of patients treated with glucocorticoids and cyclophosphamide had remission, prolonged delay in achieving remission and relapses led in both patient groups to freedom from active disease for approximately 50% of the total patient-years. As a result, morbidity was substantial and has led to comparative studies of alternative therapies.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Child
  • Cyclophosphamide / adverse effects
  • Cyclophosphamide / therapeutic use
  • Eye Diseases / physiopathology
  • Female
  • Follow-Up Studies
  • Glomerulonephritis / physiopathology
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / physiopathology*
  • Humans
  • Laryngostenosis / physiopathology
  • Lung Diseases / physiopathology
  • Male
  • Nose Diseases / physiopathology
  • Opportunistic Infections
  • Prednisone / adverse effects
  • Prednisone / therapeutic use
  • Prospective Studies
  • Remission Induction
  • Survival Rate
  • Treatment Outcome


  • Cyclophosphamide
  • Prednisone