Wegener's granulomatosis in a child

A post-mortem diagnosis of Wegener's granulomatosis (WG) was made on a ten-year-old girl. The patient had presented with recurrent sinusitis, progressive proteinuria, and reduced renal function. Biopsies from maxillary sinuses had revealed non-specific chronic inflammation, and a renal biopsy extracapillary glomerulonephritis. Chest X-ray had shown a cavitating nodule in the right lung, initially interpreted as tuberculosis. Lung biopsy had not been performed. To achieve a precise diagnosis in cases with symptoms suggesting WG, open lung biopsy, and abundant biopsy specimens from involved organs are advocated.