I also continue prednisone 5 mg/day for a year in most patients, regardless of their induction regimen or ANCA specificity. I stress that expert opinion varies widely on this issue. It is again appropriate to involve the patient in the decision making in light of the uncertainties. Parenthetically, many patients, particularly those with GPA and upper-airway disease, require low or moderate doses of prednisone and other immune-suppressive drugs to keep inflammation in check on a chronic basis; this issue is distinct from “remission maintenance” as I have discussed it.
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A final area of controversy in the management of patients with AAV in remission is the value of ANCA titers. A recent meta-analysis of studies of cANCA or anti-PR3 titers (there is much less data on anti-MPO) concluded that a rise in ANCA titer (two- to fourfold, or transition from negative to positive, in different studies) is associated with an approximately twofold increase in risk of flare, and that persistence of ANCA following induction therapy is associated with a smaller increase in risk.17 It is therefore not surprising that there is difference of opinion in how to approach changes in titer clinically. I admit that I am always gratified to see an ANCA titer drop to a normal or very low level after induction therapy and am relieved when it remains there during or after maintenance therapy, but I do not escalate or reinitiate maintenance therapy when a titer rises, nor do I base the choice or duration of maintenance drug(s) on ANCA titers.
Although there remain many uncertainties about the optimal approach(es) to induction and maintenance therapy in AAV, the good news for patients and providers is that multiple options are supported by the available evidence, and that national and international collaborations will ensure that the scientific basis for more effective and/or less toxic therapies will continue to expand.
Dr. Monach is assistant professor in the section of rheumatology and director of the Vasculitis Center at Boston University School of Medicine.
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- Pagnoux C, Hogan SL, Chin H, et al. Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: Comparison of two independent cohorts. Arthritis Rheum. 2008;58:2908-2918.
- De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461-2469.
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- Stone JH, Merkel PA, Seo P, et al. Extended follow-up of treatment with rituximab versus cyclophosphamide for remission-induction of ANCA-associated vasculitis: Which subsets are at greatest risk for flare? Arthritis Rheum. 2011;63:S946.
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- Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. 1996;335:16-20.
- de Groot K, Reinhold-Keller E, Tatsis E, et al. Therapy for the maintenance of remission in sixty-five patients with generalized Wegener’s granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum. 1996;39:2052-2061.
- Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349:36-44.
- Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790-2803.
- Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: A randomized controlled trial. JAMA. 2010;304:2381-2388.
- Metzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener’s granulomatosis. Rheumatology (Oxford). 2007;46:1087-1091.
- Wegener’s Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med. 2005;352:351-361.
- Walsh M, Merkel PA, Mahr A, Jayne D. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis. Arthritis Care Res (Hoboken). 2010;62:1166-1173.
- Jones RB, Smith R, Guerry MJ, et al. Protocolized versus non-protocolized rituximab treatment for refractory ANCA-associated vasculitis. Arthritis Rheum. 2010;62:S283.
- Cartin-Ceba R, Golbin J, Keogh KA, et al. Rituximab for remission induction and mainenance in ANCA-associated vasculitis: A single-center ten-year experience in 108 patients. Arthritis Rheum. 2010;62:S283.
- Tomasson G, Grayson PC, Mahr AD, Lavalley M, Merkel PA. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis—a meta-analysis. Rheumatology (Oxford). 2012;51:100-109.