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Eosinophilic Granulomatosis with Polyangiitis (EGPA / Churg-Strauss)

Rare ANCA-associated small-vessel vasculitis with eosinophilia, asthma, and multi-organ involvement — pulmonary, neurologic, cardiac, cutaneous, and renal — highly responsive to glucocorticoids and biologic therapy.

Written by: Saygı Hospital Health Guide Editorial Board
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This content is for general information; please consult your physician for diagnosis and treatment.

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What is Eosinophilic Granulomatosis with Polyangiitis (EGPA / Churg-Strauss)?

EGPA is a rare necrotizing small- and medium-vessel vasculitis characterized by eosinophil-rich and granulomatous inflammation. It belongs to ANCA-associated vasculitides (AAV) but only ~30–40% of patients are MPO-ANCA positive (typically those with renal/peripheral nerve involvement). The remainder are ANCA-negative and have predominant cardiac/pulmonary disease.

ACR/EULAR 2022 classification: physician-diagnosed asthma, eosinophilia (>1×10⁹/L or >10%), extravascular necrotizing granuloma, neuropathy (mono/polyneuropathy), pulmonary infiltrates, paranasal sinus abnormality, hematuria, and absence of MPO-ANCA. Modified Five-Factor Score predicts mortality based on cardiac, GI, renal, age, and CNS involvement.

Three clinical phases: prodromal (years to decades) — asthma, allergic rhinitis, nasal polyposis; eosinophilic — peripheral eosinophilia, pulmonary infiltrates, eosinophilic gastroenteritis; vasculitic — mononeuritis multiplex, cardiomyopathy (most common cause of death), purpura, glomerulonephritis.

Symptoms

Severe adult-onset asthma, often steroid-dependent, refractory to standard therapy
Allergic rhinitis, chronic rhinosinusitis with nasal polyps
Peripheral eosinophilia (>10% or >1500/μL absolute count)
Migratory pulmonary infiltrates on imaging
Mononeuritis multiplex (foot drop, wrist drop) — high specificity
Skin manifestations: palpable purpura, nodules, livedo, cutaneous ulceration
Cardiac: myocarditis, pericarditis, cardiomyopathy, conduction abnormalities (~50% involvement, leading cause of mortality)
Gastrointestinal: eosinophilic gastroenteritis, abdominal pain, GI bleeding
Renal: glomerulonephritis (less common than other AAV)
Constitutional: fever, weight loss, fatigue, myalgia, arthralgia

Risk Factors

Pre-existing severe asthma and atopy
Chronic rhinosinusitis with nasal polyps
Aspirin-exacerbated respiratory disease
Leukotriene receptor antagonist use (controversial — may unmask vs. cause)
Mepolizumab/omalizumab tapering of systemic corticosteroids
MPO-ANCA positivity (renal/neuropathy phenotype)
Genetic associations: HLA-DRB4, IL-5 polymorphisms

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Adult-onset severe asthma with peripheral eosinophilia — pulmonologist and rheumatologist referral
  • New mononeuritis multiplex (foot drop, wrist drop) — urgent evaluation
  • Cardiac symptoms (chest pain, dyspnea, arrhythmia) in patient with eosinophilic disease
  • Palpable purpura with eosinophilia
  • Renal abnormalities (hematuria, proteinuria) in asthmatic patient

Treatment Methods

01
Diagnostic workup: complete blood count with eosinophilia, MPO-ANCA, IgE, complement, CRP, urinalysis, chest CT, echocardiography (mandatory due to high cardiac risk), cardiac MRI for myocardial involvement, EMG/NCS for neuropathy, biopsy of affected tissue (skin, sural nerve, kidney, lung) when feasible
02
Risk stratification (FFS 2009): cardiac, GI, age >65, renal, CNS involvement → 1 point each; FFS ≥1 indicates higher mortality and need for cyclophosphamide
03
Induction therapy: high-dose corticosteroids (oral prednisolone 1 mg/kg/day, IV pulse methylprednisolone 1 g/day × 3 in severe disease) plus cyclophosphamide (oral or IV) for organ-threatening disease (FFS ≥1) or rituximab as alternative for MPO-ANCA positive patients
04
Mild disease (FFS = 0): corticosteroids alone or with methotrexate/azathioprine for steroid-sparing
05
Maintenance therapy: azathioprine, methotrexate, mycophenolate, or rituximab; avapritinib for tyrosine kinase mutations in selected eosinophilic patients
06
Mepolizumab (anti-IL-5, 300 mg SC monthly): first FDA-approved biologic for EGPA, reduces relapses and corticosteroid dose, especially effective in non-severe disease and asthma control
07
Reslizumab and benralizumab (other anti-IL-5 agents) under investigation
08
Cardiac involvement: cardiology co-management, anticoagulation if endomyocardial fibrosis or mural thrombus, heart failure therapy
09
Mononeuritis multiplex: physical and occupational therapy, pain management with gabapentin/pregabalin, IVIG in selected cases
10
Asthma control: high-dose ICS-LABA, leukotriene modifiers (caution interpretation), biologics (mepolizumab, dupilumab in some cases)
11
Long-term monitoring: ANCA titers, eosinophil count, organ function, prevention of relapses, screening for treatment toxicity (osteoporosis, infections, malignancy)

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.