Patient handout

Eosinophilic granulomatosis with polyangiitis (EGPA)

PRODUCTION

1. Your condition

This handout is for eosinophilic granulomatosis with polyangiitis (egpa). Your care team identified this based on: late-onset / refractory asthma + chronic rhinosinusitis + new systemic features.

Other reasons your team may use this plan: marked peripheral eosinophilia (>1.5 x10^9/l or >10%) with multisystem disease; mononeuritis multiplex / asymmetric sensorimotor neuropathy (foot/wrist drop); new cardiomyopathy / myocarditis or migratory pulmonary infiltrates in an asthmatic.

2. Your medications

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

Medication	Starting dose	How	When	What it does
Five-Factor Score (FFS 2011) + BVAS stratification	—	—	—	2011 FFS revision — FFS 0 routes to non-severe (GC + mepolizumab); FFS ≥1 or cardiac/GI/RPGN/alveolar hemorrhage/severe neuropathy routes to severe induction (CYC/RTX)

Plan: EGPA — FFS / organ-threatening severity triage (2021 ACR/VF + EULAR + MIRRA)

3. When to call your provider

Contact your care team if any of the following happen:

New cardiac symptoms / troponin rise → urgent echo + admit (eosinophilic myocarditis) (2021 ACR/VF)
New active urine sediment / rising creatinine → urgent RPGN workup + admit (2021 ACR/VF)
New mononeuritis multiplex / foot-drop → urgent NCS + escalate to severe pathway (2021 ACR/VF)
Status asthmaticus / hypoxia → ED + ICU (GINA; 2021 ACR/VF)

4. When to seek emergency care

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

Eosinophilic myocarditis / cardiomyopathy with reduced EF, restrictive physiology, malignant arrhythmia, or cardiogenic shock (rising troponin/natural marker of fluid overload (BNP), echo/CMR abnormality)(life-threatening)
Hypoxia + hemoptysis + diffuse pulmonary infiltrates + falling hemoglobin(life-threatening)
Rapidly progressive GN — rising creatinine + active urine sediment (dysmorphic RBCs / RBC casts), more frequent in the ANCA+ subset(life-threatening)
Severe / progressive mononeuritis multiplex — asymmetric sensorimotor deficit, foot-drop / wrist-drop, functionally disabling vasculitic neuropathy
Eosinophilic gastroenteritis with GI bleeding, ischemia, or perforation (FFS GI factor)(life-threatening)
Severe acute asthma exacerbation — hypoxia, silent chest, exhaustion, or near-fatal asthma in EGPA(life-threatening)

5. Follow-up

Lifelong rheumatology + pulmonology continuity; maintenance steroid-sparing agent + mepolizumab; minimize cumulative glucocorticoid; relapse surveillance (eosinophils, asthma escalation, ANCA, new neuropathy/cardiac); vaccination, bone health, cardiovascular risk reduction; benralizumab as emerging steroid-sparing option

6. Sources

Guideline: 2021 ACR/VF Vasculitis Guideline (EGPA) + 2022 ACR/EULAR EGPA classification + MIRRA mepolizumab trial; Five-Factor Score (FFS) 2011 revision