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Patient handout

Microscopic polyangiitis (MPA)

PRODUCTION

1. Your condition

This handout is for microscopic polyangiitis (mpa). Your care team identified this based on: positive mpo-anca + rising creatinine / active urine sediment.

Other reasons your team may use this plan: pulmonary-renal syndrome — hemoptysis / hypoxia + rpgn; dysmorphic rbcs / rbc casts + rising cr (pauci-immune rpgn); fever / weight loss + palpable purpura + mononeuritis multiplex.

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
renal biopsyprocedure2021 ACR/VF — pauci-immune necrotizing crescentic GN; distinguish from anti-GBM linear IgG and immune-complex GN; % normal glomeruli prognosticates renal recovery
plasma exchange (selective)extracorporeal7 exchanges over 14 days (typical)PEXIVAS — NOT routine; selective for dialysis-dependent / severe AKI from GN or DAH; standard of care if concomitant anti-GBM

Plan: MPA — severity triage → induction (GC PEXIVAS taper + RTX/CYC ± PEX ± avacopan) → prophylaxis → maintenance → ILD → relapse/ESKD (2021 ACR/VF + KDIGO 2024)

3. When to call your provider

Contact your care team if any of the following happen:

  • New active sediment / rising Cr → urgent renal workup + re-induction (KDIGO 2024 ANCA GN)
  • Hemoptysis / hypoxia → ED for DAH (2021 ACR/VF)
  • New mononeuritis multiplex / major relapse → expedited inpatient re-induction (2021 ACR/VF)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Hypoxia + hemoptysis + diffuse infiltrates + falling Hb (pulmonary capillaritis)(life-threatening)
  • Rapidly progressive crescentic GN with dialysis-dependent AKI(life-threatening)
  • ANCA-positive AND anti-GBM-positive (double-positive disease)(life-threatening)
  • Rapidly progressive fibrotic MPA-ILD with hypoxemic respiratory failure(life-threatening)
  • Asymmetric motor/sensory deficit — vasculitic mononeuritis multiplex

5. Follow-up

Prolonged maintenance (rituximab or azathioprine) with relapse surveillance; ESKD pathway and kidney transplant timing (defer until sustained remission); MPA-ILD progression monitoring ± antifibrotic; long-term CV risk modification, infection prophylaxis, malignancy surveillance (CYC bladder/hematologic); vaccination (avoid live vaccines on immunosuppression); fertility/family planning

6. Sources

Guideline: 2021 ACR/VF Vasculitis Guideline (ANCA-associated) + KDIGO 2024 ANCA GN + 2022 ACR/EULAR classification + PEXIVAS/ADVOCATE/RAVE

  1. pubmed.ncbi.nlm.nih.gov/34235894
  2. pubmed.ncbi.nlm.nih.gov/32053298
  3. pubmed.ncbi.nlm.nih.gov/33596356