Microscopic polyangiitis
ICD-10 M31.7 · ICD-11 4A44.A0

Treatment of Non-Organ-Threatening MPA When Initial Induction Therapy Has Failed

This protocol applies to patients with microscopic polyangiitis (MPA) without organ-threatening or life-threatening disease manifestations who have not achieved or sustained remission following structured first-line induction therapy.

Clinical Scenario Non-organ-threatening and non-life-threatening microscopic polyangiitis with persistent or recurring disease activity despite prior remission-induction attempts.
Prior Therapy — Failure Condition First-line induction used oral prednisolone combined with rituximab — or, as alternatives, methotrexate, mycophenolate mofetil, or avacopan — along with trimethoprim-sulfamethoxazole prophylaxis and maintenance therapy with rituximab, azathioprine, or methotrexate. The expected goals of remission within 4–6 months and sustained remission over 24–48 months of maintenance were not achieved, prompting escalation to this protocol.
Approach (Partial Overview) Management starts with a thorough reassessment of disease status and comorbidities; the full protocol specifies which immunosuppressive options and referral pathways apply in this refractory setting.

Complete treatment selection and sequencing are available in the structured regimen below.

Treatment Goal Remission — defined as absence of typical signs, symptoms, or other features of active ANCA-associated vasculitis.
Instant Access to Structured Evidence-Based Regimens

References

  1. 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.
  2. For patients with GPA or MPA with disease refractory to therapy to induce remission, we recommend a thorough reassessment of disease status and comorbidities and consideration of options for additional or different treatment.
  3. These patients should be managed in close conjunction with, or referred to, a centre with expertise in vasculitis.
  4. It may be considered for remission induction in non-organ-threatening disease when the alternatives RTX, MTX and MMF cannot be used or are ineffective.
  5. Adding intravenous immunoglobulins can be an option for persistent disease manifestations, particularly in patients with increased risk of infection.
DOI: 10.1136/ard-2022-223764 View source ↗