Organ-Threatening Microscopic Polyangiitis When Initial Induction Therapy Did Not Achieve Remission
This protocol applies to patients with organ-threatening or life-threatening microscopic polyangiitis (MPA) whose disease has not responded adequately to first-line induction treatment.
Microscopic polyangiitis with organ-threatening or life-threatening disease manifestations. Current evidence-based recommendations call for induction with glucocorticoids combined with rituximab or cyclophosphamide as the first step for patients in this category.
First-line induction for organ-threatening MPA involves glucocorticoids in combination with rituximab or cyclophosphamide. The targets this line must achieve are:
• Remission at 6 months — defined as absence of typical signs, symptoms, or other features of active ANCA-associated vasculitis
• Sustained remission throughout the maintenance period
When these targets are not met, escalation to the next-line protocol is indicated.
Management of refractory disease begins with a thorough reassessment of disease status and comorbidities; the protocol then specifies adjustments to the immunosuppressive approach and the role of specialist referral.
The overarching goal remains remission: absence of typical signs, symptoms, or other features of active ANCA-associated vasculitis.