Late recurrence of Wegener's granulomatosis presenting as tracheal stenosis in a renal transplant patient

An unusual case of a 46-year-old man who had end-stage renal disease due to crescenlic glomerulonephritis that was treated by a cadaveric renal allograft is presented. About 18 months post-Tx, the patient developed severe tracheal stenosis which, on biopsy, revealed granulation tissue and chronic inflammation. After another 18 months he developed necrotizing glomerulonephritis involving the renal allograft and an inflammatory retro-orbital pseudotumor. The latter, on biopsy, revealed granulomatous vasculitis characteristic of Wegener's granulomatosis. This diagnosis was further confirmed by strongly positive anti-ncutrophil antibodies with diffuse granular cytoplasmic immunofluorescence (C-ANCA). The patient was treated successfully with cyclophosphamide therapy.