Wegener granulomatosis in children and adolescents: Clinical presentation and outcome

https://doi.org/10.1016/S0022-3476(05)83482-1Get rights and content

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 malignancles 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 achleving 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.

References (17)

There are more references available in the full text version of this article.

Cited by (225)

  • Vasculitis and Kidney Disease

    2022, Pediatric Clinics of North America
    Citation Excerpt :

    The disease is characterized by necrotizing granulomatous lesions of the respiratory tract, necrotizing vasculitis involving both small- and medium-sized vessels, and focal necrotizing glomerulonephritis. Clinically, upper and lower respiratory tract involvement in the form of recurrent sinusitis (most common), recurrent epistaxis, chronic otitis media, conductive hearing loss, or subglottic stenosis are the most common presenting symptoms.42 Asymptomatic to fulminant pulmonary involvement in the form of stridor, hoarseness of voice, cough, dyspnea, pleuritic chest pain, and hemoptysis are commonly reported.43

  • Overview of Pediatric Rheumatology: Part Two

    2021, Physician Assistant Clinics
  • 58 - Pulmonary Involvement in the Systemic Inflammatory Diseases of Childhood

    2019, Kendig's Disorders of the Respiratory Tract in Children
  • Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case–control study from the French Vasculitis Study Group Registry

    2018, Autoimmunity Reviews
    Citation Excerpt :

    However, those findings are of limited value because they were derived from studies from different specialties (nephrology, pulmonology, rheumatology), and used different disease classification criteria. To the best of our knowledge, only one study, published in the early 1990s and limited to GPA patients, compared some aspects of c- and aGPAs [14], but most questions remain unanswered. This study was designed to compare the initial characteristics and subsequent evolutions of cAAV patients vs. matched aAAV controls prospectively enrolled in the French Vasculitis Study Group (FVSG) Registry.

  • Childhood systemic vasculitis

    2017, Best Practice and Research: Clinical Rheumatology
View all citing articles on Scopus
View full text