Endobronchial vasculitis in childhood granulomatosis with polyangiitis

Laryngo-tracheo-bronchial disease in childhood granulomatosis with polyangiitis may acutely present with endobronchial small vessel vasculitis without airway stenosis. Treatment should not be delayed in the presence of haemoptysis as it may indicate acute pulmonary capillaritis which can lead to fatal respiratory failure from diffuse alveolar haemorrhage.