Prognosis

Prior to the introduction of effective treatment, patients with untreated systemic granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis) had a median survival of 5 months and most died within 12 months. Now, with aggressive immunosuppressive regimens, remission can be achieved in the large majority of patients. However, despite these advances, disease- and treatment-related morbidity and mortality remains a major problem.[69][70] In one study, mortality was 13.6% at 1 year and increased with age.[7] One third to one half of all patients will suffer at least one disease relapse.[9] Furthermore, the vast majority of patients will develop at least one complication related to GPA and/or its treatment.[71] Data from a large multicentre cohort study suggest that the main causes of mortality are infection, cardiovascular disease, and active vasculitis.[72]

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