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Over the last decades, numerous randomised clinical trials and long-term observational studies were conducted in dozens or hundreds patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and provided a basis for an evidence-based treatment. With immunosuppressive therapy, up to 85%–90% of patients will achieve remission, though the disease can follow a relapsing course in a significant proportion of patients, and prolonged exposure to glucocorticoids and immunosuppressive agents may have devastating consequences. However, the attempts to shorten maintenance therapy can lead to recurrent exacerbations of AAV and an accumulation of irreversible organ damage.
In the recent randomised, prospective REMAIN (prolonged REmission-MAINtenance therapy in systemic vasculitis) study, conducted in 117 patients with AAV in stable remission after cyclophosphamide/prednisolone-based induction, Karras et al showed that prolonged maintenance therapy with azathioprine/prednisolone (up to 48 months from diagnosis) was relatively safe and more effective than withdrawal of azathioprine/prednisolone by 24 months1. Extension of immunosuppression was associated with a significantly lower risk both of any and major relapses (22% vs 63%, p<0.0001, and 14% vs 35%, p<0.007, respectively) and resulted in a better renal survival. Notably, almost all patients completed the prolonged follow-up. The results of the REMAIN study were satisfying to us since prolonged immunosuppression with low dose cyclophosphamide and currently azathioprine or metothrexate in combination with corticosteroids for many years remains a preferred treatment option for patients with AAV in our clinic.
However, there is the other side of the coin. A paradigm for treating patients with AAV has changed significantly over recent years. Currently, it is apparent that …