Wegener-Granulomatose im Kindes- und Jugendalter

Wegener’s granulomatosis (WG) is a necrotising granulomatous small vessel vasculitis with a clinical predilection for the involvement of the upper airways, lungs and kidneys. It occurs at all ages. The pathogenesis of WG is determined by the pathological activation of phagocytes during transmigration through the vessel. Whereas most aspects of WG are similar at all ages, some features appear to be significantly different. WG in childhood is more frequently complicated by subglottic stenosis and nasal deformity, while treatment related morbidity is less common compared to adults. The introduction of combined treatment with cyclophosphamide and glucocorticoids has resulted in a dramatic improvement in patient outcome; however, commonly occurring disease relapses and the risk of chronic organ damage at all ages make long-term follow-up of all patients necessary.