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rheum.egpa.core.v1

Eosinophilic granulomatosis with polyangiitis (EGPA)

rheumatologyacutechronicadultacuteinpatientoutpatient
Start encounter →Print handoutPRODUCTION

Manifest pointer prisma/seed/manifests/rheum.gca.chronic.v1.ts is a PLACEHOLDER — no EGPA-specific manifest authored yet; atoms/phenotypes/regimen drug list require manifest backing (tracked in design brief). No problem-package folder under src/lib/tier3/problem-package/packages/ for EGPA — design brief authored, atoms pending. regimen_axes intentionally carry NO rxcui — RxNav validation deferred to PRODUCTION (mepolizumab, benralizumab, cyclophosphamide, rituximab, prednisone, methylprednisolone, MTX, AZA, TMP-SMX backfill pending). Five-Factor Score (FFS 2011) + BVAS are referenced as the risk-stratification surface; no dedicated calc_ffs / calc_bvas adapter in clinical-tools-registry.ts — only generic calc.news2 / calc.qsofa used. workup.polyarthritis reused as the systemic-vasculitis INITIAL_WORKUP surface; workup.rpgn / workup.acute_weakness cover the vasculitic GN / mononeuritis-multiplex branches. Bayesian likelihood ratios for eosinophilia / ANCA / asthma toward EGPA vs HES/ABPA/parasitic deferred to a future evidence pass.

Entry points (5)

  • symptom
    Late-onset / refractory asthma + chronic rhinosinusitis + new systemic features
    adult_asthma_eosinophilia_systemic
  • lab_abnormality
    Marked peripheral eosinophilia (>1.5 x10^9/L or >10%) with multisystem disease
    marked_peripheral_eosinophilia
  • symptom
    Mononeuritis multiplex / asymmetric sensorimotor neuropathy (foot/wrist drop)
    mononeuritis_multiplex
  • symptom
    New cardiomyopathy / myocarditis or migratory pulmonary infiltrates in an asthmatic
    eosinophilic_cardiac_pulmonary
  • problem_list
    Known EGPA with rising eosinophils / asthma escalation / new organ involvement (relapse)
    known_egpa_relapse

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Age >65 is a Five-Factor Score (FFS) point; gonadotoxicity counselling before cyclophosphamide is age/fertility dependent
  • asthma_historyrequired
    symptom • used at ENTRY
    Late-onset / difficult-to-control asthma is a 2022 ACR/EULAR classification criterion and near-universal in EGPA
  • sinonasal_diseaserequired
    symptom • used at CONTEXT
    Chronic rhinosinusitis / nasal polyposis is prodromal; ABSENCE of ENT disease is an adverse FFS factor
  • absolute_eosinophil_countrequired
    lab • used at INITIAL_WORKUP
    Peripheral eosinophilia >1.5 x10^9/L (or >10% WBC) is the strongest 2022 ACR/EULAR criterion and the key relapse biomarker
  • anca_mpo_pr3required
    lab • used at INITIAL_WORKUP
    Only ~30-40% ANCA+ (usually p-ANCA/anti-MPO); ANCA+ subset is more vasculitic (GN, PNS) — pivots induction choice
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Eosinophilic myocarditis is the leading cause of EGPA death and is frequently clinically silent — screen even when asymptomatic
  • creatininerequired
    lab • used at INITIAL_WORKUP
    RPGN / pauci-immune GN is an organ-threatening FFS feature; drives cyclophosphamide/rituximab induction and renal dosing
  • urinalysis_with_microrequired
    lab • used at INITIAL_WORKUP
    Active sediment / dysmorphic RBCs / RBC casts signal vasculitic GN — escalates to severe-disease pathway
  • organ_systems_involvedrequired
    symptom • used at RISK_STRATIFICATION
    Cardiac / GI / renal / severe PNS / alveolar hemorrhage define organ-threatening disease and FFS — drive treatment intensity
  • echocardiogram
    imaging • used at BRANCHING_WORKUP
    Eosinophilic cardiomyopathy (reduced EF, restrictive physiology, mural thrombus) mandates urgent immunosuppression + cardiology
  • current_immunosuppressionrequired
    medication • used at CONTEXT
    Baseline glucocorticoid dose / mepolizumab / steroid-sparer adherence; abrupt steroid taper can unmask vasculitic phase
  • pregnancy_or_fertility_status
    history • used at CONTEXT
    Cyclophosphamide is gonadotoxic — fertility preservation / GnRH-agonist protection counselling required before induction

12-phase flow (12)

  1. 1FRAME
    Asthmatic adult with eosinophilia + multisystem disease — apply 2022 ACR/EULAR EGPA classification only after mimics excluded; new diagnosis vs known-EGPA relapse
    inputs: asthma_history
    advance: EGPA scope confirmed; phase (prodromal / eosinophilic / vasculitic) characterized
  2. 2ENTRY
    Recognize trigger: late-onset/refractory asthma + sinonasal disease + new systemic feature, marked eosinophilia, mononeuritis multiplex, or cardiopulmonary involvement
    inputs: asthma_history, organ_systems_involved
    advance: presenting phenotype documented
  3. 3CONTEXT
    Capture age, sinonasal history, current asthma controller + glucocorticoid dose, mepolizumab/steroid-sparer use, recent steroid taper, leukotriene-receptor-antagonist exposure, fertility/pregnancy status, infection screen
    inputs: age, sinonasal_disease, current_immunosuppression, pregnancy_or_fertility_status
    advance: baseline therapy, precipitants, and comorbidities captured
  4. 4RED_FLAGS
    Eosinophilic myocarditis / cardiogenic shock (leading killer), diffuse alveolar hemorrhage, rapidly progressive GN, severe mononeuritis multiplex, eosinophilic GI perforation/bleed, status asthmaticus → ICU + pulse methylprednisolone
    inputs: troponin, creatinine, organ_systems_involved
    actions: calc.news2
    advance: organ-threatening red flags screened and acted on; ICU triggered if present
  5. 5INITIAL_WORKUP
    CBC with differential (absolute eosinophil count), ANCA (MPO/PR3 + IF), troponin, BNP, ECG, CMP, UA + micro, ESR/CRP, total IgE, IgG4, stool O&P + Strongyloides serology (pre-immunosuppression), CXR, CT chest
    inputs: absolute_eosinophil_count, anca_mpo_pr3, troponin, creatinine, urinalysis_with_micro
    actions: workup.polyarthritis, panel.cbc, panel.inflammation, panel.cardiac, panel.renal, panel.ua
    advance: eosinophil count, ANCA, cardiac + renal screen, parasite screen, and chest imaging resulted
  6. 6BRANCHING_WORKUP
    Echo + cardiac MRI if troponin/BNP up or symptomatic (eosinophilic cardiomyopathy); RPGN workup + renal biopsy if active sediment; nerve conduction studies + nerve/muscle biopsy for mononeuritis multiplex; tissue biopsy (extravascular eosinophils, necrotizing vasculitis, granuloma); bronchoscopy/BAL if alveolar hemorrhage
    inputs: echocardiogram
    actions: workup.rpgn, workup.acute_weakness, panel.cmp, panel.coag
    advance: organ-specific confirmatory workup booked / resulted
  7. 7DIFFERENTIAL
    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
    inputs: absolute_eosinophil_count, anca_mpo_pr3
    advance: mimics excluded; 2022 ACR/EULAR classification applied
  8. 8RISK_STRATIFICATION
    Five-Factor Score 2011 revision (cardiac involvement, GI involvement, renal insufficiency Cr >1.7 mg/dL, age >65, ABSENCE of ENT manifestations) — FFS 0 = non-organ-threatening; FFS ≥1 or cardiac/GI/RPGN/alveolar hemorrhage/severe neuropathy = organ/life-threatening; BVAS for activity; ANCA+ vs ANCA- phenotype
    inputs: organ_systems_involved, age
    actions: calc.qsofa
    advance: FFS computed, BVAS scored, severity tier (non-severe vs severe/organ-threatening) set
  9. 9TREATMENT
    Glucocorticoid backbone for all. Non-severe (FFS 0, no organ-threatening): GC + mepolizumab (anti-IL-5, MIRRA — steroid-sparing + relapse reduction) ± methotrexate/azathioprine. Severe / organ-threatening / cardiac / alveolar hemorrhage / RPGN / severe neuropathy (FFS ≥1): pulse methylprednisolone + GC + cyclophosphamide OR rituximab induction. Treat asthma per GINA. Cardiac EGPA: urgent immunosuppression + HF/arrhythmia management + cardiology. Supportive: PJP prophylaxis, bone protection, vaccination, fertility preservation pre-CYC. Avoid abrupt GC taper (vasculitic-phase unmasking)
    inputs: organ_systems_involved, anca_mpo_pr3, creatinine
    advance: induction regimen + asthma control + prophylaxis + maintenance plan documented
  10. 10DISPOSITION
    Cardiac EGPA / alveolar hemorrhage / RPGN / GI ischemia / status asthmaticus → ICU. Organ-threatening (FFS ≥1) → admit with urgent rheumatology + organ-specialist co-management. Non-severe → expedited rheumatology / pulmonology outpatient escalation
    inputs: organ_systems_involved
    advance: level of care set; multidisciplinary consults secured
  11. 11MONITORING
    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
    inputs: absolute_eosinophil_count, troponin, creatinine
    actions: panel.cbc, panel.cardiac, panel.renal
    advance: remission achieved; transition to maintenance (steroid-sparer + mepolizumab)
  12. 12FOLLOWUP
    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
    advance: long-term maintenance + relapse-surveillance plan documented