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Wegener’s granulomatosis is a systemic vasculitis mainly affecting the lungs, nasal sinuses, and kidneys. Treatment usually consists of cyclophosphamide (Cy) and steroids.1 High dose Cy with autologous stem cell support could be an alternative treatment for patients resistant to conventional treatment or requiring long term immunosuppression with the risk of secondary malignancy.2
CASE REPORT
We report on a 33 year old woman with chronic relapsing sinusitis, pulmonary granuloma, and proteinuria with progressive renal insufficiency since 1988. Renal biopsy showed necrotising glomerulonephritis, and biopsy of the nasal sinus showed granuloma with necrotising vasculitis. Proteinase-3-antineutrophil cytoplasmic antibodies (PR3-ANCA) were detectable with a titre of 1/280. Despite treatment with Cy (750 mg/m2 every 3 weeks, later 100 mg/day orally) and steroids renal function deteriorated, and she underwent dialysis from April 1995 to December 1998. Live kidney transplantation from the patient’s sister matched …
Footnotes
The first and last authors contributed equally to this work.