Treatment of Microscopic Polyangiitis with Organ-Threatening or Life-Threatening Disease Manifestations

This protocol covers patients with microscopic polyangiitis (MPA) presenting with organ-threatening or life-threatening disease manifestations — a high-acuity subset requiring prompt induction therapy to avert irreversible damage.

Induction of remission in this setting involves a combination of glucocorticoids together with an immunosuppressive agent. The specific agent selected and the broader management strategy depend on clinical features and the individual disease course.

The complete regimen — including agent selection, dosing strategy, sequential adjustments, and maintenance — is detailed in the full protocol.

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