Maintenance Therapy for ANCA-Associated Vasculitis

Guideline-Based Recommendations

The Kidney Disease: Improving Global Outcomes (KDIGO) guidelines recommend maintenance therapy with either rituximab or azathioprine and low-dose glucocorticoids after induction of remission, with a Grade 1C recommendation 1
The American College of Rheumatology/Vasculitis Foundation guidelines conditionally recommend rituximab over methotrexate or azathioprine for remission maintenance in patients with severe GPA/MPA whose disease entered remission after treatment with cyclophosphamide or rituximab 2

The optimal duration of remission therapy is between 18 months and 4 years after induction of remission, according to the KDIGO guidelines 1, 3, 4, 5
Acceptable rituximab maintenance dosing protocols include the MAINRITSAN scheme: 500 mg × 2 at complete remission, then 500 mg at months 6, 12, and 18 thereafter 3

The KDIGO guidelines recommend that glucocorticoids should be continued at 5-7.5 mg/day for 2 years and then slowly reduced by 1 mg every 2 months during maintenance therapy, but also note that rituximab can be used as monotherapy 1, 3
The KDOQI US Commentary notes that the role of low-dose prednisone for maintaining remission has not been rigorously tested and cannot be universally recommended 6

The 2021 ACR/VF guidelines conditionally recommend scheduled re-dosing over using ANCA titers or CD19+ B cell counts to guide re-dosing 2, 7
Rituximab without glucocorticoids has been used successfully for maintenance therapy following cyclophosphamide induction 6

Stopping maintenance therapy before 18 months significantly increases relapse risk, according to the KDIGO guidelines 1, 4, 5
Patients with severe disease characteristics, such as renal and pulmonary involvement, require more prolonged maintenance therapy 1, 3