Summary

Head and neck manifestations are very common in WG and may result in significant morbidity. WG is commonly associated with PR3-ANCA and is highly responsive to treatment with GC and cytotoxic agents. Modern therapy has reduced mortality from WG, from 50% within five months to less than 20% over at least eight years of follow-up. However, disease- and treatment-related morbidity is considerable. The notion that current therapies cure WG has been abandoned for the more realistic view that therapy is life-saving and converts a usually fatal disease to a chronic one.

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