The underlying pathology behind antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is not clear; more effective and safer treatments are needed. High-dose glucocorticoids, cyclophosphamide, other autoimmune drugs are routinely used to reduce inflammation, and plasma exchange is used to rapidly remove ANCAs. In order to investigate the effectiveness of high-dose glucocorticoids and plasma exchange, the international PEXIVAS randomized trial recruited 704 patients with severe ANCA-associated vasculitis. Patients were followed for a median of 2.9 years. A reduced-dose regime of glucocorticoids was noninferior to a standard, high-dose regime; death or severe end-stage kidney disease was similar in both groups (92/330 [27.9%] in the reduced-dose group vs. 83/325 (25.5%) in the standard, high-dose group; absolute risk difference, 2.3 percentage points; 90% CI, -3.4 to 8.0). Patients in the reduced-dose glucocorticoids group had fewer serious infections compared to the high-dose group. In addition, plasma exchange did not reduce the hazard of death or severe end-stage kidney disease for patients in the plasma exchange group compared to the control group (28.4% vs. 31%, respectively; hazard ratio, 0.86; 95% CI, 0.65-1.13). Plasma exchange, however, may be beneficial to specific subgroups of patients, including those with ANCA-induced pulmonary hemorrhage. Further research and new treatment options are needed for patients with severe ANCA-associated vasculitis.
References:
- Walsh M, Merkel PA, Peh CA, Szpirt WM, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. The New England Journal of Medicine 2020; 382 (7); 622-631.
- Derebail VK and RJ Falk. ANCA-associated vasculitis—refining therapy with plasma exchange and glucocorticoids. The New England Journal of Medicine 2020; 382 (7); 671-673.
