Mild presentations may be adequately treated with corticosteroids alone. Many patients, however, cannot be tapered to low, minimally toxic doses of prednisone and require a second agent. MTX, used as has been described for milder forms of WG, may be adequate for these purposes. MTX is relatively contraindicated in patients with renal impairment. In such cases, alternative therapies that may be of value include AZA or mycophenolate mofetil. There is limited published experience regarding any of these agents in CSS. More severe cases require dual therapy with GC and CYC. Relapses requiring retreatment are common. Disease severity, as determined by organ system involvement and rate of progression, dictates choices of therapy.

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