Treatment of Microscopic Polyangiitis Without Organ-Threatening or Life-Threatening Disease Manifestations

Clinical Scenario

This protocol applies to patients with microscopic polyangiitis (MPA) who present without organ-threatening or life-threatening disease manifestations. This sub-population is distinct from more severe presentations and is managed with an induction regimen calibrated to this lower-severity context, followed by structured maintenance.

Treatment Goals

The primary target is remission at 4–6 months, defined as the absence of typical signs, symptoms, or other features of active ANCA-associated vasculitis. The sustained goal is maintained remission over 24–48 months on maintenance therapy.

Approach Overview

Induction of remission involves a combination of glucocorticoids and a targeted biologic agent, with alternative immunosuppressive agents available when the primary option is not suitable. The protocol additionally specifies a glucocorticoid reduction strategy, infection prophylaxis considerations, and a defined maintenance phase — the full regimen, individualized dosing approach, and sequencing are contained in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2022-223764

For induction of remission of non-organ-threatening or non-life-threatening GPA or MPA, treatment with a combination of glucocorticoids and rituximab is recommended.

Methotrexate or mycophenolate mofetil can be considered as alternatives to rituximab.

For patients with AAV receiving rituximab, cyclophosphamide and/or high doses of glucocorticoids, we recommend the use of trimethoprim–sulfamethoxazole as prophylaxis against Pneumocystis jirovecii pneumonia and other infections.

For maintenance of remission of GPA and MPA, after induction of remission with either rituximab or cyclophosphamide, we recommend treatment with rituximab. Azathioprine or methotrexate may be considered as alternatives.

We recommend that therapy to maintain remission for GPA and MPA be continued for 24–48 months following induction of remission of new-onset disease.

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