However, it is necessary Fedratinib mouse to reduce the dose of cyclophosphamide for patients with advanced
renal dysfunction. 3. Maintenance therapy of ANCA-positive RPGN Cyclophosphamide along with azathioprine, mizoribine, mycophenolate mofetil and methotrexate have been reported as immunosuppressants in patients with AAV. Treatment with azathioprine or mizoribine in patients with ANCA-positive RPGN is recommended as maintenance therapy to prevent a relapse. Bibliography 1. Koyama A, et al. Clin Exp Nephrol. 2009;13:633–50. (Level 4) 2. Ozaki S, et al. Mod Rheumatol. 2012;22(3):394–404. (Level 4) 3. Jayne D, et al. N Engl J Med. 2003;349:36–44. (Level 2) 4. Hirayama K, et al. Am J Kidney Dis. 2004;44:57–63. (Level 5) 5. Hiemstra TF, et al. JAMA. 2010;304:2381–8. (Level 2) 6. Langford CA, et al. Arthritis Rheum. 1999;42:2666–73. (Level 3) 7. Langford CA, et al. Am J Med. 2003;114:463–9. (Level 4) Is the addition of plasmapheresis to treatment recommended in patients with RPGN? Treatment with immunosuppressive therapy plus plasmapheresis has improved the outcome of patients with RPGN. Prospective studies in patients with ANCA-associated vasculitis (AAV) and retrospective studies in patients with anti-GBM antibody-positive RPGN have been performed in
European countries selleck screening library and the US. 1. ANCA-positive RPGN ANCA is thought to be involved in the clinical conditions of AAV and RPGN. The removal of ANCA may, therefore, result in controlling disease activity and preventing organ damage. Addition of plasmapheresis to the initial therapy with corticosteroids
and cyclophosphamide is indicated for patients presenting with either advanced kidney failure (sCr >5.8 mg/dl) or with diffuse alveolar hemorrhage. 2. Anti-GBM antibody-positive RPGN We recommend plasmapheresis to improve the outcome of patients with anti-GBM antibody-positive RPGN. On the other hand, in patients with advanced kidney failure or dialysis, there is only rare evidence that plasmapheresis improves the outcome. 3. Immune C1GALT1 complex RPGN We recommend plasmapheresis for patients with immune complex RPGN, while considering the patient’s age, organ damage and pathological findings. Bibliography 1. Jayne DR, et al. J Am Soc Nephrol. 2007;18:2180–8. (Level 2) 2. Szpirt WM, et al. Nephrol Dial Transplant. 2011;26:206–13. (Level 2) 3. Walters GD, et al. BMC Nephrol. 2010;11:12. (Level 1) 4. Walsh M, et al. Am J Kidney Dis. 2011;57:566–74. (Level 1) 5. Akt inhibitor Yamagata K, et al. J Clin Apher. 2005;20:244–51. (Level 4) 6. Cui Z, et al. Medicine (Baltimore). 2011;90:303–11. (Level 4) 7. Flores JC, et al. Lancet 1986;1:5–8. (Level 5) Are corticosteroids recommended for maintenance therapy in patients with RPGN? After remission due to the initial treatment, maintenance therapy is needed to prevent a relapse.