Sclerosing mediastinitis, granulomatous glomerulonephritis, and systemic vasculitis in a child: a unique association

The association of sclerosing mediastinitis, granulomatous glomerulonephritis and systemic vasculitis found at necropsy of a 9-year-old girl is presented. The histologic findings strongly suggested that the vasculitis played a central role in the development of the sclerosing mediastinitis, which was not associated with granulomas in the mediastinal lymph nodes. The combination of glomerulonephritis, systemic vasculitis, and terminal diffuse pulmonary hemorrhage with acute capillaritis suggests a link between this case and Wegener's granulomatosis, although mediastinitis has not been reported in that disease.