Juvenile idiopathic arthritis and Wegener's granulomatosis--causal or casual acquaintances?

We report two cases of long-term juvenile idiopathic arthritis which developed recurrent subglottic stenosis secondary to localized Wegener's granulomatosis. In both cases, the biopsies taken from the affected region shown granulomatous vasculitis with multinucleated giant cells. Both patients presented positive serology for antineutrophil cytoplasmic antibodies by immunofluorescence (c-ANCA) and ELISA (PR3). The involvement or other organs was ruled out at the time of Wegener's granulomatosis diagnosis and during follow-up. To our knowledge, this is the first report of the association between juvenile idiopathic arthritis and a localized form of Wegener's granulomatosis.