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rheum.egpa.core.v1PRODUCTION
rheum.egpa.core.v1

Eosinophilic granulomatosis with polyangiitis (EGPA)

rheumatologyacutechronicadult
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Asthmatic adult with eosinophilia + multisystem disease — apply 2022 ACR/EULAR EGPA classification only after mimics excluded; new diagnosis vs known-EGPA relapse

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Advance rule
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Advance when

EGPA scope confirmed; phase (prodromal / eosinophilic / vasculitic) characterized

Patient inputs (12)

Age >65 is a Five-Factor Score (FFS) point; gonadotoxicity counselling before cyclophosphamide is age/fertility dependent

Chronic rhinosinusitis / nasal polyposis is prodromal; ABSENCE of ENT disease is an adverse FFS factor

Baseline glucocorticoid dose / mepolizumab / steroid-sparer adherence; abrupt steroid taper can unmask vasculitic phase

Late-onset / difficult-to-control asthma is a 2022 ACR/EULAR classification criterion and near-universal in EGPA

Peripheral eosinophilia >1.5 x10^9/L (or >10% WBC) is the strongest 2022 ACR/EULAR criterion and the key relapse biomarker

Only ~30-40% ANCA+ (usually p-ANCA/anti-MPO); ANCA+ subset is more vasculitic (GN, PNS) — pivots induction choice

Eosinophilic myocarditis is the leading cause of EGPA death and is frequently clinically silent — screen even when asymptomatic

RPGN / pauci-immune GN is an organ-threatening FFS feature; drives cyclophosphamide/rituximab induction and renal dosing

Active sediment / dysmorphic RBCs / RBC casts signal vasculitic GN — escalates to severe-disease pathway

Cardiac / GI / renal / severe PNS / alveolar hemorrhage define organ-threatening disease and FFS — drive treatment intensity

Eosinophilic cardiomyopathy (reduced EF, restrictive physiology, mural thrombus) mandates urgent immunosuppression + cardiology

Cyclophosphamide is gonadotoxic — fertility preservation / GnRH-agonist protection counselling required before induction

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationallife_threateningeosinophilic_myocarditis_cardiogenic
    Eosinophilic myocarditis / cardiomyopathy with reduced EF, restrictive physiology, malignant arrhythmia, or cardiogenic shock (rising troponin/BNP, echo/CMR abnormality)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningdiffuse_alveolar_hemorrhage
    Hypoxia + hemoptysis + diffuse pulmonary infiltrates + falling hemoglobin
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningrpgn_vasculitic_gn
    Rapidly progressive GN — rising creatinine + active urine sediment (dysmorphic RBCs / RBC casts), more frequent in the ANCA+ subset
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsevere_eosinophilic_gi_disease
    Eosinophilic gastroenteritis with GI bleeding, ischemia, or perforation (FFS GI factor)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningstatus_asthmaticus
    Severe acute asthma exacerbation — hypoxia, silent chest, exhaustion, or near-fatal asthma in EGPA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_mononeuritis_multiplex
    Severe / progressive mononeuritis multiplex — asymmetric sensorimotor deficit, foot-drop / wrist-drop, functionally disabling vasculitic neuropathy
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

EGPA — FFS / organ-threatening severity triage (2021 ACR/VF + EULAR + MIRRA)
axis: egpa_severity_ffs_basedstep 1 - Severity triage — FFS 0 / non-organ-threatening vs FFS ≥1 / organ- or life-threatening
Selected step "Severity triage — FFS 0 / non-organ-threatening vs FFS ≥1 / organ- or life-threatening" — Diagnosis established (2022 ACR/EULAR) + mimics excluded — stratify by Five-Factor Score (cardiac, GI, renal Cr >1.7, age >65, ENT-absence) and organ-threatening features
  • Five-Factor Score (FFS 2011) + BVAS stratification
    first line
    risk_assessment
    triggers: diagnosis_established
    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)

outpatient playbook — drug actions (4)

  1. 1. prednisone
    0.5–1 mg/kg/day PO then taper • PO • daily, taper
    trigger: Non-severe EGPA / relapse
    GC backbone; avoid abrupt taper (2021 ACR/VF)
  2. 2. mepolizumab
    300 mg SC q4 weeks • SC • q4 weeks
    trigger: Non-severe / relapsing / steroid-dependent
    Steroid-sparing + relapse reduction (MIRRA NEJM 2017)
  3. 3. methotrexate or azathioprine
    MTX 15–25 mg weekly + folate OR AZA 2 mg/kg/day • PO/SC • weekly / daily
    trigger: Steroid-sparing maintenance
    Non-severe steroid-sparer (2021 ACR/VF)
  4. 4. ICS-LABA (GINA)
    Per GINA step • inhaled • daily
    trigger: Active asthma
    Asthma control (GINA)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Late-onset / refractory asthma + chronic rhinosinusitis + new systemic features; Marked peripheral eosinophilia (>1.5 x10^9/L or >10%) with multisystem disease; Mononeuritis multiplex / asymmetric sensorimotor neuropathy (foot/wrist drop).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Eosinophilic granulomatosis with polyangiitis (EGPA)** (rheum.egpa.core.v1).
Phenotype framing: Distinguish from hypereosinophilic syndrome (no asthma/sinusitis/vasculitis, FIP1L1-PDGFRA, end-organ eosinophil damage), parasitic infection (Strongyloides — must exclude before steroids), drug-induced eosinophilia/DRESS, ABPA (Aspergillus-specific IgE/IgG), chronic eosinophilic pneumonia, GPA/MPA (no asthma, higher ANCA-positivity), eosinophilic leukemia, lymphoma
Scope: Asthmatic adult with eosinophilia + multisystem disease — apply 2022 ACR/EULAR EGPA classification only after mimics excluded; new diagnosis vs known-EGPA relapse

No severity triggers fired against current inputs.

Plan

Regimen axis: **EGPA — FFS / organ-threatening severity triage (2021 ACR/VF + EULAR + MIRRA)** — step "Severity triage — FFS 0 / non-organ-threatening vs FFS ≥1 / organ- or life-threatening".
1. Five-Factor Score (FFS 2011) + BVAS stratification (risk_assessment, first line) — 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)

Setting playbook (outpatient) — Manage non-severe EGPA (FFS 0) and relapse with glucocorticoid + mepolizumab ± steroid-sparer, optimize asthma control, exclude organ-threatening features, expedite escalation if they emerge
2. prednisone 0.5–1 mg/kg/day PO then taper PO daily, taper — Non-severe EGPA / relapse (GC backbone; avoid abrupt taper (2021 ACR/VF))
3. mepolizumab 300 mg SC q4 weeks SC q4 weeks — Non-severe / relapsing / steroid-dependent (Steroid-sparing + relapse reduction (MIRRA NEJM 2017))
4. methotrexate or azathioprine MTX 15–25 mg weekly + folate OR AZA 2 mg/kg/day PO/SC weekly / daily — Steroid-sparing maintenance (Non-severe steroid-sparer (2021 ACR/VF))
5. ICS-LABA (GINA) Per GINA step inhaled daily — Active asthma (Asthma control (GINA))

Non-pharmacologic actions:
- Inactivated vaccination (pneumococcal, influenza, COVID, recombinant zoster)
- Bone protection if chronic glucocorticoid
- Cardiovascular risk reduction
- Relapse-surveillance education (rising eosinophils, asthma escalation, new neuropathy/cardiac symptoms)

AVOID / contraindication checks:
- Cyclophosphamide gonadotoxic — fertility counsel + gamete cryopreservation or GnRH agonist protection before induction (2021 ACR/VF)
- Cyclophosphamide give with MESNA + hydration to prevent hemorrhagic cystitis (2021 ACR/VF)
- Avoid abrupt glucocorticoid taper — may precipitate / unmask the vasculitic phase (leukotriene receptor antagonist unmasking debate noted — association now attributed to steroid withdrawal rather than causal)
- Live vaccines contraindicated on immunosuppression (2021 ACR/VF)
- Rituximab HBV screen before dosing (2021 ACR/VF)
- Azathioprine TPMT testing before initiation (2021 ACR/VF)
- Exclude Strongyloides before high dose steroids (hyperinfection risk in eosinophilia)
- PJP prophylaxis on prednisone >=20 mg >=4 weeks or CYC/RTX (2021 ACR/VF)

Monitoring

Regimen monitoring:
- absolute eosinophil count + asthma control as relapse biomarkers (MIRRA; 2021 ACR/VF)
- ANCA/MPO titer trend (ANCA+ subset relapse correlation) (2021 ACR/VF)
- serial troponin + echo if cardiac involvement (eosinophilic cardiomyopathy surveillance)
- CBC nadir 7-14 days during cyclophosphamide; cumulative CYC dose tracking (2021 ACR/VF)
- renal panel + UA on induction (vasculitic GN surveillance) (2021 ACR/VF)
- BVAS activity + VDI damage index trend (EULAR)
- infection surveillance during combined immunosuppression (2021 ACR/VF)
- glucocorticoid-toxicity (glucose, BP, bone, cataract) + cardiovascular risk surveillance

Setting (outpatient) monitoring:
- Eosinophil count + asthma control q1–3 months (MIRRA)
- ANCA, CBC, CMP, creatinine, UA q1–3 months (2021 ACR/VF)
- Troponin/echo if any cardiac symptom (2021 ACR/VF)
- BVAS / clinical activity at each visit (EULAR)

Follow-up plan: 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
- Close-out criterion: long-term maintenance + relapse-surveillance plan documented

Monitoring phase: Eosinophil count + asthma control + ANCA titer as relapse biomarkers; serial troponin + echo if cardiac involvement; CBC nadir on cyclophosphamide; ESR/CRP, renal panel, UA on induction; BVAS/VDI trend; glucocorticoid-toxicity, infection, and cardiovascular surveillance

Disposition

Current setting: outpatient — Manage non-severe EGPA (FFS 0) and relapse with glucocorticoid + mepolizumab ± steroid-sparer, optimize asthma control, exclude organ-threatening features, expedite escalation if they emerge

Disposition criteria:
- Continue outpatient unless organ-threatening feature emerges (2021 ACR/VF)

Escalation triggers (move to higher acuity):
- 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)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Eosinophilic myocarditis / cardiomyopathy with reduced EF, restrictive physiology, malignant arrhythmia, or cardiogenic shock (rising troponin/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

Citations

- 2021 ACR/VF Vasculitis Guideline (EGPA) + 2022 ACR/EULAR EGPA classification + MIRRA mepolizumab trial; Five-Factor Score (FFS) 2011 revision [PMID:34235894](https://pubmed.ncbi.nlm.nih.gov/34235894/)
- Cited evidence (PMID 35106968) [PMID:35106968](https://pubmed.ncbi.nlm.nih.gov/35106968/)
- Cited evidence (PMID 28514601) [PMID:28514601](https://pubmed.ncbi.nlm.nih.gov/28514601/)
- Cited evidence (PMID 21200183) [PMID:21200183](https://pubmed.ncbi.nlm.nih.gov/21200183/)
- Cited evidence (PMID 38393328) [PMID:38393328](https://pubmed.ncbi.nlm.nih.gov/38393328/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2021 ACR/VF Vasculitis Guideline (EGPA) + 2022 ACR/EULAR EGPA classification + MIRRA mepolizumab trial; Five-Factor Score (FFS) 2011 revision — PMID:34235894
  • Cited evidence (PMID 35106968) — PMID:35106968
  • Cited evidence (PMID 28514601) — PMID:28514601
  • Cited evidence (PMID 21200183) — PMID:21200183
  • Cited evidence (PMID 38393328) — PMID:38393328