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What Is Proper Treatment for Wegener Granulomatosis?

Ken-ichiro Inoue, MD; Toshikazu Yoshikawa, MD, PhD; Yutaka Kawahito, MD, PhD; Hajime Sano, MD, PhD
Arch Intern Med. 2001;161(14):1777-1778. doi:.
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Previously in the ARCHIVES, we reported a case of late recurring Wegener granulomatosis (WG) that was successfully treated with combination therapy consisting of cyclophosphamide and cyclosporin.1 The 36-year-old woman, whose WG recurred 18 years after her first remission with cyclophosphamide and corticosteroid therapy, was treated with cyclosporin and prednisolone after undergoing a resection of corneal granuloma. However, because of severe tracheal stenosis with subglottic granuloma, cyclophosphamide was added to her treatment regimen, and with the combination therapy of cyclophosphamide, cyclosporin, and prednisolone, her condition improved rapidly. Cyclophosphamide therapy was discontinued in January 2000 (8 months after the initiation), because of the patient's clinical stability and her rejection of cyclophosphamide therapy. In October 2000, she was referred to our clinic with hematuria, and subsequent evaluation resulted in a diagnosis of urinary bladder cancer. The patient underwent total cystectomy in February 2001. The treatment for this refractory and clinical malignant disease is difficult, and to date, cyclophosphamide is the most reliable agent against WG. However, considering that WG often affects middle-aged patients2 and that cyclophosphamide has a variety of acute and chronic complications, cyclophosphamide therapy is not recommended for all patients with WG. We report here a second patient with recurrent WG (after spontaneous abortion) who was successfully treated with cyclosporin.

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