Wegener's granulomatosis in children

A 13-year-old girl developed suppurative rhinitis responsible for necrosis of the nasal septum. Six months later, she started experiencing arthritis i n the interphalangeal joints of her right foot, then severe pneumonia with multiple nodules, She was admitted after failing several courses of antibio tics. Her laboratory tests showed marked inflammation, Wegener's granulomat osis was considered. Tests for antineutrophil cytoplasmic antibody (ANCA) w ere negative on two occasions. Diffuse nodular densities were seen on compu ted tomography scans of the chest, A surgical biopsy of Fowler's segment wa s performed under thoracoscopy. The histological diagnosis was Wegener's gr anulomatosis, Cyclophosphamide and a glucocorticoid were given for two year s. At last follow-up, the outcome was favorable, the only residual abnormal ity being the nasal septum necrosis, Pediatric Wegener's granulomatosis is rare and difficult to diagnose. Histology of a pulmonary biopsy specimen is key to the diagnosis in patients with negative tests for ANCA. Pulmonary b iopsy should be considered in patients with suggestive pulmonary, articular , and ENT symptoms but negative tests for ANCA. It is essential to make the correct diagnosis because appropriate therapy, despite the risks it entail s, improves the prognosis of this disease whose natural course is frequentl y fatal.