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Patient handout

Granulomatosis with polyangiitis (GPA)

PRODUCTION

1. Your condition

This handout is for granulomatosis with polyangiitis (gpa). Your care team identified this based on: refractory sinusitis / bloody nasal crusting / septal perforation / saddle-nose / new hearing loss.

Other reasons your team may use this plan: hemoptysis / hypoxia + active urinary sediment (pulmonary-renal syndrome); positive c-anca / anti-pr3 (or mpo) with multisystem disease; rising creatinine + dysmorphic rbcs / rbc casts (rpgn pattern).

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
tissue biopsy (renal / lung / ENT)—procedural—KDIGO 2024 — least-invasive accessible-site biopsy confirms pauci-immune necrotizing crescentic GN or necrotizing granulomatous inflammation; do not delay empiric induction in fulminant DAH/RPGN

Plan: GPA — severity triage → remission induction → adjunct prophylaxis → maintenance → relapse surveillance (2021 ACR/VF + KDIGO 2024 + PEXIVAS/ADVOCATE)

3. When to call your provider

Contact your care team if any of the following happen:

  • New organ-threatening feature (hemoptysis, rising Cr, stridor, foot drop) → urgent admission (2021 ACR/VF)
  • Rising PR3-ANCA + ENT recurrence → intensify surveillance / consider re-induction (2021 ACR/VF)
  • Severe infection on immunosuppression → hold ISD, ID evaluation (2021 ACR/VF)

4. When to seek emergency care

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

  • Hemoptysis + hypoxia + diffuse alveolar infiltrates + falling Hb (DAH)(life-threatening)
  • Rising creatinine + RBC casts → pauci-immune crescentic GN; oliguric/dialysis-dependent AKI(life-threatening)
  • Stridor / progressive dyspnea from subglottic or tracheobronchial stenosis(life-threatening)
  • Cerebral vasculitis / pachymeningitis / seizure or myocarditis-pericarditis-coronary involvement(life-threatening)
  • Acute foot/wrist drop or asymmetric sensorimotor deficit — vasculitic neuropathy

5. Follow-up

Prolonged maintenance (RTX redosing schedule or AZA/MTX) ≥18–48 mo with relapse surveillance; manage accrued damage (VDI) — CKD, hearing loss, subglottic stenosis, nasal deformity; cardiovascular + venous-thromboembolism + infection + malignancy (CYC bladder) long-term risk; vaccination catch-up; bone health; fertility/pregnancy planning when stable

6. Sources

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

  1. pubmed.ncbi.nlm.nih.gov/34235894
  2. pubmed.ncbi.nlm.nih.gov/35106964
  3. pubmed.ncbi.nlm.nih.gov/32053298