This protocol covers patients with granulomatosis with polyangiitis (GPA) who present without organ-threatening or life-threatening manifestations and in whom the goals of the first treatment line — achieving and sustaining remission — have not been met.
The first line combined prednisolone with rituximab (or methotrexate or mycophenolate mofetil as alternatives) and targeted two goals: complete remission — defined as absence of typical signs, symptoms, or other features of active disease — and sustained remission throughout 24–48 months of maintenance. When either or both of these goals are not reached, escalation to this protocol is indicated.
Patients have non-organ-threatening or non-life-threatening GPA whose disease has proved refractory to standard induction and maintenance strategies. A thorough reassessment of both disease status and comorbidities is the essential starting point before any further treatment decision.
When rituximab, methotrexate, and mycophenolate mofetil have each proven insufficient or cannot be used, an alternative immunosuppressive agent — cyclophosphamide — may be considered. There are also additional options for specific patient profiles, and management in close conjunction with a centre experienced in vasculitis is a core part of the approach. The full decision framework and criteria for each option are in the protocol.
Target: Remission — absence of signs, symptoms, or features of active diseaseDOI: 10.1136/ard-2022-223764