ANCA-associated vasculitis: mission incomplete

Over the last decades, introduction of high-dose corticosteroids and immunosuppressive agents and later rituximab into the current algorithms for remission induction and maintenance treatment resulted in a tremendous improvement in the survival of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). However, in the recent meta-analysis of observational studies, Tan et al showed a 2.7-fold increased risk of death in patients with AAV when compared with the general population.1 Notably, there was a trend towards lower mortality in the most recent compared with the earlier cohorts. In our own study in 242 patients with granulomatosis with polyangiitis, we also found a significant decrease in mortality in the recent years (2004–2012 vs 1970–2003; p=0.04) and a shift towards a higher percentage of cardiovascular events and complications of immunosuppression as the causes of death.2

The results of Tan et al’s meta-analysis are not surprising and suggest that AAV, particularly if not promptly diagnosed and treated, remains a life-threatening disease and requires proper management. The pitfalls of the current treatment for AAV are well known and include relatively frequent relapses, especially in proteinase-3 (PR3) ANCA-positive patients (up to 50% within 5 years), delayed diagnosis and late initiation of treatment in a proportion of patients, high rate of end-stage renal disease (ESRD), which did not change significantly in the current era, unknown optimal duration of maintenance therapy, burden of immunosuppression (eg, infections and malignancy), and increased risk of cardiovascular and thromboembolic events, which may be related …

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