Although the mortality of Wegener granulomatosis (WG) has been decreased significantly by treatment with cyclophosphamide, recurrence is also widely reported. Cytoplasmic pattern antineutrophil cytoplasmic antibody (C-ANCA) is considered an important marker for demonstrating the disease's activity. Therefore, if WG does not flare-up on long-term follow-up of more than 15 years and if C-ANCA and other inflammatory signs do not relate to clinical manifestations, it may be difficult to understand the condition of the patient and to treat the disease when it recurs. We present a case of WG manifesting 2 necrotic lesions and 1 granulomatous lesion (ie, oral mucosa, cornea, and trachea after an 18-year period of clinical stability).
Inoue K, Kondo M, Inoue M, Ishino H, Kamitsuji Y, Sano H. Successful Treatment With Combination Therapy of Cyclophosphamide and Cyclosporin for Late Recurrence of Wegener Granulomatosis. Arch Intern Med. 2000;160(3):393-394. doi: