Treatment of Granulomatosis with Polyangiitis with Organ-Threatening or Life-Threatening Disease
When granulomatosis with polyangiitis (GPA) presents with organ-threatening or life-threatening manifestations, prompt induction of remission is the clinical priority, followed by sustained maintenance therapy.
Clinical Scenario
This protocol addresses patients with granulomatosis with polyangiitis who have organ-threatening or life-threatening disease manifestations — a presentation requiring active, structured induction of remission.
Treatment Approach
Induction of remission involves glucocorticoids combined with an immunosuppressive agent — agent selection, escalation criteria, maintenance strategy, and infection prophylaxis are covered in the full structured protocol.
Treatment Goals
The primary goal is remission — absence of typical signs, symptoms, and features of active disease — with the aim of sustained remission throughout an extended maintenance period.
References
DOI: 10.1136/ard-2022-223764
- For induction of remission in patients with new-onset or relapsing GPA or MPA with organ-threatening or life-threatening disease, we recommend treatment with a combination of glucocorticoids and either rituximab or cyclophosphamide.
- Remission: Absence of typical signs, symptoms, or other features of active AAV with or without immunosuppressive therapy.
- Sustained remission: Absence of typical signs, symptoms, or other features of active AAV over a defined time period with or without immunosuppressive therapy.
- 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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