Evidence-based treatment of ANCA-associated vasculitis with kidney involvement

Download in PDF

ANCA-associated vasculitides (AAV) is a group of rare systemic autoimmune disorders characterized by inflammation of the small blood vessels in different organs. Kidney involvement is one the most common and severe features of AAV. The combination of immunosuppressive medications, predominantly cyclophosphamide and rituximab, with corticosteroids (CS) remains the standard of care for remission induction. The results of the PEXIVAS trial showed that redu-ced dose CS regimen was noninferior to the standard regimen. The results of two randomized trials suggested that mycophenolate mofetil can be used for remission induction in patients with non-life-threatening AAV. The role of plasma exchange in the treatment of AAV remains unclear, since it did not improve overall or renal survival in the PEXIVAS trial. The most well studied medications for maintenance therapy include azathioprine and rituximab. The results of randomized trials (MAINRITSAN, RITAZAREM) showed the superiority of rituximab over azathioprine in terms of relapse-free survival. The optimal duration of maintenance therapy is undefined and probably should be no less than 18 months. However, some patients might benefit from extended maintenance therapy of up to 48 months. Adverse effects of immune suppressive therapy, e.g. infectious diseases, malignancies, osteoporosis and venous thromboembolic events, are common and often require active prophylaxis.

Key words

Antineutrophil cytoplasmic antibodies, ANCA-associated vasculitis, rapidly progressive glomerulonephritis, corticosteroids, cyclophosphamide, rituximab, mycophenolate mofetil, plasma exchange, azathioprine.