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.