Recurrent Wegener's granulomatosis: A case report and review

Background: Wegener's granulomatosis is a granulomatous systemic necrotizing vasculitis with prominent upper airway involvement. Complete remissions can be induced with aggressive management. Despite successful treatment, relapses and recurrences of active disease may occur. Objective: We present a patient who presents with histologically confirmed active disease 17 years following successful therapy. Methods: A case report of a 72-year-old man who initially presented at age 50 with necrotizing vasculitis of the upper and lower respiratory tract. Eighteen years following his initial therapy he presented with isolated retroorbital disease. Following re-institution of cytotoxic therapy he appears to be disease-free. Results: Following re-treatment with cytoxan and prednisone, a second remission was induced. The patient is currently entirely well 1 year off therapy. Conclusion: Wegener's granulomatosis may recur many years following apparent successful and aggressive cytotoxic and anti-inflammatory therapy.