How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy

Abstract

Recent progress has notably improved outcomes for patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), namely granulomatosis with polyangiitis and microscopic polyangiitis. Since 2021, several international scientific societies have recommended rituximab (RTX) as the preferred primary treatment for maintaining remission in AAV patients. Decisions regarding retreatment with RTX are based on individual patient risk factors for disease flare-ups and the potential consequences of such flares. In reviewing available evidence and reporting our experiences at G. Bosco Hub Hospital in Turin, Italy, we explore various trials focusing on the maintenance therapy in AAV and discuss areas of unmet need.

Keywords: ANCA vasculitis; maintenance therapy; off therapy; relapse; rituximab; stopping therapy.

Figure 1

The algorithm proposed by San G. Bosco Hub Hospital in Turin focuses on ANCA specificity and status. In our Turin cohort, a persistently negative ANCA test, particularly with MPO specificity, has demonstrated a strong negative predictive value for relapse. The assessment of histological activity through repeated renal biopsies is considered when there is a re-emergence or exacerbation of urinary abnormalities. Abbreviations: +ve, positive; −ve, negative.