rheum.reviews

Eosinophilic granulomatosis and polyangiitis (EGPA)

Contents
    Author: Dr. Yan Yeung
    Reviewer: Dr. Lillian Barra
    Dr. Yan Yeung, MD FRCPC:
    Assistant Professor (Adjunct), Department of Medicine, Division of Rheumatology
    McMaster University

    Dr. Lillian Barra, MD MPH FRCPC:
    Associate Professor, Department of Medicine Division of Rheumatology,
    Department of Microbiology and Immunology,
    Department of Epidemiology and Biostatistics
    Western University

    Topic last updated: July 2025
    Topic last reviewed: July 2025

    BOTTOM LINE

    EGPA vasculitis (formerly “Churg-Strauss Syndrome”) is a small/medium vessel necrotizing granulomatous systemic vasculitis characterized by asthma and chronic rhinosinusitis with blood and tissue eosinophilia; systemic symptoms may include petechiae, neuropathy and gastroenteritis. Cardiac involvement is an important cause of death and patients should be screened for cardiac involvement. pANCA (anti-MPO antibodies) are positive in 30-40% of patients.  All patients are induced with glucocorticoids; cyclophosphamide is used in those with poor prognostic factors as assessed by the Five Factor Score. Mepolizumab amongst other therapies are used for maintenance.
    [🕑 10 minute read ]

    EPIDEMIOLOGY

    Epidemiology not well known; 10x less common than GPA and MPA

    • Prevalence
      • ~2-22/1,000,000
    • Incidence
      • 0.5-3.7/1,000,000 annually
    • Age, Sex
      • Age: mean age at onset 40-60 years; uncommon >65 years and in children.
      • Poor prognosis if onset ≥65 years.
      • Sex: equal M=F predominance
    • Risk factors
      • Genetic
        • MHC-associated:
          • HLA-DRB1*04, HLA-DRB1*07, HLA-DRB4
          • HLA-DQ associated with anti-MPO positive EGPA but not anti-MPO negative EGPA
        • Non-MHC associated:
          • IL-10.2 haplotype of IL-10 gene, variants at TSLP, BCL2L11, CDK6, BACH2, Chromosome 10, and LPP loci
      • Environmental
        • Silica dust, organic solvents, and farming
        • Smoking may be associated with lower risk.

    CLINICAL FEATURES

    — CLINICAL PHASES

    • Prodrome
      • Allergic disease; precedes eosinophilic and vasculitic phase by 8-10 years.
      • Asthma (96-100%), fevers (50%), atopy, allergic rhinitis
    • Eosinophilic
      • Peripheral and infiltrative tissue eosinophilia of multiple organs
      • Migratory lung opacities/eosinophilic pneumonia, eosinophilic gastroenteritis, +/- myocarditis
      • Variable period, up to 8-10 years.
    • Vasculitis
      • Asthma may abate; patients develop sometimes life-threatening vasculitis which can cause myocarditis, vasculitic neuropathy, glomerulonephritis, gastroenteritis, petechiae/purpura
      • May be associated with ANCA
    Clinical phases can overlap; some patients lack ‘eosinophilic’ or ‘vasculitic’ manifestations entirely
    Patients do not necessarily linearly follow sequence of stages
    Some manifestations (ex: thrombotic events) have neither eosinophilic or vasculitic mechanism

    — IMMUNOPHENOTYPES

    Two main overlapping immunophenotypes of EGPA based on ANCA positivity
    ANCAs positive in 30-40%, usually anti-MPO

    Common to ANCA-positive or -negative EGPA

    • Eosinophilia
    • Chronic sinusitis with nasal polyposis
    • Asthma

    ANCA-Positive EGPA: More frequently associated with vasculitic features:

    • HLA-DRB4, DRB1, IL-10.2 haplotype association.
    • Petechiae/purpura.
    • Neuropathy.
    • Glomerulonephritis.
    • Diffuse alveolar hemorrhage.

    ANCA-negative EGPA: More frequently associated with eosinophilic features:

    • Nasal polyposis.
    • Lung infiltrates– eosinophilic pneumonia.
    • Gastroenteritis.
    • Cardiomyopathy.
    • IL-5 upregulation.

    — ORGAN MANIFESTATIONS.

    • Upper airway
      • Rhinosinusitis (73%), nasal polyposis (53%), otitis media (14%), nasal crusting (15%)
      • Compared to GPA:
        • EGPA should not cause destructive necrotizing sinusitis with saddle nose and otitis media
    • Lower Airway
      • Asthma (90-100%): adult onset or asthma worsening in adulthood; can precede vasculitis ~ 8-10 years
      • Pulmonary infiltrates (50%): migratory or peri-bronchovascular patchy lesions including ground glass changes, bronchial wall thickening, “tree-in-bud” sign, consolidation, and non-cavitating nodules
      • Pleural effusions (9%, usually eosinophilic)
      • Alveolar hemorrhage (4%; more common if ANCA+)
      • Interstitial lung disease (6%)
    • Cardiovascular
      • Cardiac involvement occurs early in disease, more common in EGPA (30-45%) than in GPA/MPA
      • Cardiomyopathy (16%), pericarditis (15%), myocarditis, pericardial effusion, dysrhythmias, coronary arteritis
      • Associated with higher peripheral eosinophilia
      • MRI reveals late myocardial gadolinium enhancement (sensitive finding)
      • Venous thromboembolism (8%)

    Cardiovascular complications are a common cause of death in EGPA

    • Hematological
      • Peripheral eosinophilia (>90%); mean 7.4±6.4×109/L
      • Predates systemic manifestations by ~1.5 years
    • Skin
      • Petechiae/purpura (22%), tender subcutaneous granulomatous nodules, livedo, urticaria, necrotic lesions
    • Neurological
      • Peripheral neuropathy (of any kind 44-73%):
        • Mononeuritis multiplex, manifesting as wrist/foot drop, sensory deficits, neuropathic pain (46%)
      • Central nerve system involvement rare (5%):
        • Ischemic lesions, hemorrhage, cranial neuropathy
      • Ophthalmic:
        • CRAO, CRVO, ischemic optic neuropathy, conjunctival nodules, orbital myositis, proptosis, dacroadenitis, retinal vasculitis, blindness, temporal arteritis
    • Renal
      • Renal involvement 25% (less common than in GPA [70-80%], MPA [80%])
      • Ranges from isolated proteinuria/microscopic hematuria without renal insufficiency to acute kidney injury and GN
      • Biopsies reveal pauci-immune necrotizing GN; may also show interstitial nephritis, membranous nephropathy, membranoproliferative GN
    • Gastrointestinal
      • GI involvement (any kind) ~20%
      • Eosinophilic gastroenteritis, abdominal pain, nausea, vomiting, diarrhea
      • “Surgical abdomen” — ischemic bowel (6%), GI bleed (3%)
    • Musculoskeletal
      • Arthralgias, inflammatory arthritis, rare myositis

    INVESTIGATIONS

    • CBC
      • Eosinophilia (>1500/uL or >10% total leukocyte count); can be missed if already on steroids
      • Degree of eosinophilia can correspond to disease activity
    • ALT, CK
      • Usually normal
    • Creatinine, urinalysis
      • Creatinine may be elevated, may have proteinuria/hematuria/casts
    • ESR, CRP
      • Elevated, non-specific
    • IgE, IgG, IgA, IgM
      • IgE high (70%)
      • Hypergammaglobulinemia (IgG/A/M, reflecting inflammation)
    • IgG4
      • Serum IgG4 subtype elevated in 75% of active EGPA and is more reflective of disease activity than IgE.
    • C3, C4
      • Normal
    • Cardiac
      • Serum
        • Troponin, NT-proBNP ; high peripheral eosinophilia predicts cardiac involvement
      • Echocardiogram
        • Wall motion abnormalities, evidence of myo/pericarditis, pericardial effusion, mural/valvular thrombi
      • Cardiac MRI
        • Consider if abnormal Echo/ECG; late gadolinium-enhancement may suggest cardiomyopathy for biopsy
        • Late-gadolinium lesions can also seen in asymptomatic patients with otherwise normal cardiac work-up

    Screening ECG and echocardiogram at diagnosis; involvement is a leading cause death

    • Antibodies
      • ANCA
        • ANCA 30-40%, usually anti-MPO pANCA. Anti-PR3 is unusual.
        • If ANCA+, higher risk renal disease, alveolar hemorrhage, mononeuritis, purpura
      • Rheumatoid factor
        • Positive 36-50%
    • Respiratory
      • X-ray chest
        • Migratory patchy consolidations, ground glass attenuation, diffuse opacities, nodules
        • Pleural effusions (exudative and eosinophilic)
      • CT chest
        • Migratory or peri-bronchovascular lesions including ground glass changes, bronchial wall thickening, consolidation, and non-cavitating nodules
      • Sputum
        • May have sputum eosinophilia
      • Bronchoalveolar Lavage
        • Not routine; may be done to work up infection or alveolar hemorrhage
        • Bronchoscopy shows mucosal inflammation, mucous, granulations; BAL fluid shows eosinophilia (33%)
    • Biopsy
      • Ideal to obtain if possible; potential targets: skin, renal, sural nerve, lacrimal gland
      • General findings: small necrotizing granulomas, necrotizing vasculitis of small arteries and veins, eosinophilic infiltrates; pauci-immune GN.
    • Screening for other causes of Hypereosinophilia
      • Infectious
        • Stool cultures for parasites (ex: stronglyoides) and serology for Toxocara, stronglyoides, and screening for other parasites depending on travel/exposure history
        • Serum Aspergillus fumigatus-specific IgE and IgG, culture from sputum or BAL for Aspergillus
        • Other fungal infections – coccidiomycosis, histoplasmosis, cryptococcus
        • HIV
      • Hematological
        Suggests malignant hematological cause of hypereosinophilia:
        • Blood smear showing dysplastic eosinophils or blasts
        • FIP1L1 fusion proteins, PDGFRB or FGFR1 genes detected with FISH or RT-PCR
        • Elevated serum tryptase, Vitamin B12
        • Hepatosplenomegaly on abdominal ultrasound, anemia and/or thrombocytopenia (less specific)
        • Consider bone marrow biopsy and T cell immunophenotyping as indicated

    — INVESTIGATIONS, AS INDICATED:

    • ?Peripheral neuropathy
      • EMG: for symptomatic peripheral neuropathy to target possible sural nerve biopsy
    • ?Glomerulonephritis
      • Renal biopsy
    • ?GI bleeding, ?eosinophilic gastroenteritis, ?bowel ischemia
      • Endoscopy, CT
    • ?Eosinophilic pneumonia, ?hemoptysis/alveolar hemorrhage, ?interstitial lung disease
      • CT, BAL, transbronchial or surgical biopsy if indicated; consider infection or malignancy
    • ?Clinical signs of allergic bronchopulmonary aspergillosis
      • Aspergillus-specific IgE and/or IgG sputum (or BAL) cultures for Aspergillus spp.
    • ?Skin vasculitis
      • Skin biopsy, may be an easy accessible target if tissue required to support systemic vasculitis diagnosis
    • ?Cardiomyopathy, as suggested by clinical assessment and echocardiogram
      • Cardiac MRI
    • ?Primary hematological disorder
      • T cell immunophenotyping
      • Bone marrow biopsy

    DIAGNOSIS

    Suspect EGPA in a patient presenting with difficult-to-control/adult-onset asthma, chronic rhinosinusitis, peripheral eosinophilia, and end-organ involvement— in particular lung infiltrates, peripheral neuropathy, cardiomyopathy, gastroenteritis (pain, diarrhea, bleeding), or petechial/purpuric rash. ANCAs may be supportive, though only positive in 30-40%. Biopsy of an affected organ should be sought (commonly skin, renal, peripheral nerve, lacrimal gland) and findings may reveal tissue eosinophilia with necrotizing granulomatous inflammation. If suspected, all patients should be screened for renal involvement (creatinine,  urinalysis) and cardiac involvement (ECG, echocardiogram).

    Diagnostic evaluation of patients with suspected EGPA often benefits from a multidisciplinary approach.

    DIFFERENTIAL DIAGNOSIS

    • Allergy
      • Atopy, primary eosinophilic asthma
    • Drug
      • DRESS, other drug-reactions (ex: Aspirin-exacerbated respiratory disease: rhinosinusitis, asthma from ASA/NSAID)
    • Respiratory
      • Chronic eosinophilic pneumonia: non-granulomatous biopsy, no other organs involved, ANCA negative
      • Allergic bronchopulmonary aspergillosis (APBA): asthma + radiographic opacities, no extra-pulmonary features, positive serology and cultures for aspergillosis.
    • Hematological
      • Hyper-eosinophilic syndrome (HES), subtypes:
        • Myeloid and lymphoid neoplasms with eosinophilia;
          associated mutations in PDGFRA, PDGFRB, FGFR1
        • Chronic eosinophilic leukemia or other myeloid neoplasm with eosinophilia;
          No associated mutations
        • Lymphoproliferative HES variant;
          Aberrant IL-3/IL-5 driven eosinophilia production
        • Idiopathic HES
    • Rheumatological
      • Other AAV: GPA, MPA.
        • GPA more likely PR3+, sinusitis with destruction/necrosis, GN; no asthma nor eosinophilia
      • IgG4-related disease
        • Histology typically reveals distinct features like storiform fibrosis and obliterative phlebitis without EGPA features like vasculitis or eosinophilic granulomata
    • Infectious
      • Toxocariasis, strongyloidiasis, other parasites and fungi, HIV

    CLASSIFICATION CRITERIA

    EGPA is a clinical diagnosis. Classification criteria are not meant as diagnostic criteria to diagnose disease in a single specific patient. Classification criteria are a standardized way of recruiting a well-defined homogenous population of patients in research studies in order to ensure comparability across studies of a heterogenous disease.

    ACR/EULAR 2022 CLASSIFICATION CRITERIA~

    Apply criteria to classify a patient as having EGPA when a diagnosis of small– or medium-vessel vasculitis has already been made
    Exclude diagnoses mimicking vasculitis prior to applying criteria
    Score ≥6 is needed to classify EGPA. Sensitivity 85%, Specificity 99%

    CLINICAL CRITERIA

    Obstructive airway disease+3
    Nasal polyps+3
    Mononeuritis multiplex+1

    LABORATORY AND BIOPSY CRITERIA

    Blood eosinophil count ≥1×109/L+5
    Extravascular eosinophilic-predominant inflammation on biopsy+2
    Positive cANCA or anti-PR3 antibody-3
    Hematuria-3

    DISEASE MEASURES

    5-FACTOR SCORE (FFS)

    Revised 2009 FFS1996 FFS
    Age > 65 Cardiac involvement
    Cardiac insufficiency GI involvement
    Renal insufficiency (Cr >150umol/L)Renal insufficiency (Cr >141umol/L)
    GI involvement (bleeding, perforation, infarction, pancreatitis) Proteinuria (>1 g/day)
    Absence of ENT (presence associated with better prognosis)Central nervous system involvement
    • Presence of each factor is given one point. Higher score implies poorer prognosis
    • Also used in PAN, GPA, MPA vasculitis
    • Score ranges from 0 to 2:
      • 0 = none of the factors is present
      • 1 = one factor
      • 2 = two or more factors.

    TREATMENT

    — Treatment Goal

    Absence of clinical signs or symptoms attributable to active disease, including asthma and ENT manifestations, off prednisone or if not possible ≤7.5 mg/day.

    Risk stratify patients with Five factor score to guide treatment and clinical judgement

    — Non-severe EGPA

    NON-SEVERE EGPA: FFS = 0
    No organ/life-threatening manifestations

    INDUCTION

    • Prednisone 1mg/kg/day (max 80mg/day)
      • Taper within 2-4 weeks until 20mg/d by 3 months.
      • Taper to 5mg/d over 12-18 months. More rapid steroid taper not studied in EGPA.

    MAINTENANCE

    • Glucocorticoid taper as above
    • May add mepolizumab 300mg SC q 4 weeks
      • More effective for eosinophilia-driven manifestations
      • Note: requires higher dosing than asthma indication
    • May add conventional immunosuppressant if progressive or vasculitis-driven features even if FFS remains 0 (ex: neuropathy with functional impairment)
      • Azathioprine, methotrexate, leflunomide, MMF, or even cyclophosphamide in some cases

    — Severe EGPA

    SEVERE EGPA: FFS ≥1
    Organ/life-threatening manifestations

    INDUCTION

    • Steroids
      • Methylprednisolone 500-1000mg IV daily pulses for 3 days (unproven benefit),

        then
      • Prednisone 1mg/kg/day (max 80mg/day)
        • Taper within 2-4 weeks until 20mg/d by 3 months.
        • Taper to 5mg/d over 12-18 months. More rapid steroid taper not studied in EGPA.
    • Cyclophosphamide (CYC):
      • CYC 1.5-2mg/kg/day oral x 3 months (until remission), then 1.5mg/kg/day oral x 3 months (max 200mg); or
      • CYC 15mg/kg/day IV q2 weeks x 3, then q3 weeks x 3-6 months
      • Lifetime cumulative dose should not exceed 25g of CYC
      • CYC IV: Adjust dose for age, renal clearance:
    AgeIV CYC dose
    Creatinine 150-300 mmol/L    		IV CYC dose
    Creatinine 300-500 mmol/L
    <6015 mg/kg12.5 mg/kg
    60-7012.5 mg/kg10 mg/kg
    >7010 mg/kg7.5 mg/kg
    • CYC IV: adjust dose for age (alternate strategy)
      • Age >65: fixed dose CYC 500mg IV monthly x 6 months
    • CYC PO: adjust dose for age
      • ↓25% dose for >60 years-old
      • ↓50% dose for > 75 years-old
    • Rituximab (RTX): less evidence, reserved for patients failing CYC treatment/contraindications to CYC, especially if ANCA positive — managed at centre of expertise
      • RTX 1g IV day 0 and day 14, or
      • RTX 375mg/m2 weekly x 4

    MAINTENANCE

    • Rituximab 1g IV q6 months
    • Mepolizumab 300mg SC q4 weeks
    • Azathioprine 2mg/kg/day
    • Methotrexate 20-25mg/week
    • Leflunomide 20mg/day
    • Mycophenolate mofetil 1-2.5 g/day
    • Benralizumab 30mg q4 weeks
    • Reslizumab 3 mg/kg once every 4 weeks

    — Evaluating Treatment Response

    • Clinical monitoring to detect symptoms and signs of active disease
      • Review and bedside assessment
      • Routine labs and urinalysis with ACR
      • Periodic echocardiogram, ECG, and PFTs
      • As-indicated investigations, including imaging and EMGs
    • Biomarkers: no reliable biomarker to detect disease activity or relapse
      • Eosinophilia, CRP, IgE, serum IgG4:
        • Can be elevated with disease activity, but association may be weak
      • ANCA:
        • ANCA positivity not necessarily reflective of disease activity in all patients
        • Not recommended to re-dose rituximab maintenance based on ANCA titers
        • Value of ANCA monitoring is debated.
        • If MPO-ANCA is positive at disease onset, ANCA monitoring may detect the persistence, rise or re-appearance of ANCA and suggest patients whom require closer follow-up

    — Adjunctive management

    • PJP Prophylaxis
      • Trimethoprim-sulfamethoxazole DS 1-tab Monday/Wednesday/Friday
      • Initiation: start with induction therapy
      • Duration: at least 3 months following CYC cessation or 6 months following RTX cessation
      • If allergy or intolerance: dapsone or atovaquone as alternative
    • GI ulcer
      • Add a PPI if on high dose steroids and if other risk factors for GI ulcer
    • Screening and Vaccines
      • Bladder cancer:
        Urinalysis q3-6 months lifelong to screen for bladder malignancy if induced with CYC
      • Skin cancer:
        Routine skin exams for non-melanoma skin cancer 
      • Bone density:
        Usual screening for patients taking chronic steroids
      • Immunoglobulin levels:
        • Patients on RTX getting severe/recurrent infections
        • If hypogammaglobilinemia (ex, IgG <3g/L) before RTX dose, give supplemental immunoglobulin (multidisciplinary decision, made with Clinical Immunology)
      • Vaccinations:
        Pneumococcal, influenza, COVID, shingles vaccine for everyone (ideally 5 months post RTX infusion and 4 weeks prior to next RTX infusion)

    PROGNOSIS

    • Mortality
      • 5-year survival: 92-97% survival
      • Risk factor for mortality: Age ≥65, cardiac or renal involvement
      • Most common causes of death: cardiomyopathy, treatment-related infection
    • Relapses
      • Relapses common:
        • 41% have ≥1 relapses by ~26 months after treatment onset, usually at <10mg/d of prednisone
      • Increase risk for relapse:
        • Anti-MPO positivity (HR 2.18; 95% CI, 1.21-3.91)
        • GI involvement (HR 6.75; 95% CI 1.55-29.52 )
      • Decrease risk for relapse:
        • Baseline eosinophil >3000/mm (HR 0.50; 95% CI, 0.27-0.91)
    • Complications
      • Chronic asthma, rhinitis, neuropathic pain and functional impairment, chronic renal insufficiency, treatment-related osteoporosis, type 2 diabetes mellitus
    • Common EGPA-related causes of death
      • Infectious complications
      • Cardiac: heart failure, myocardial infarction
      • Lung disease, asthma

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    Updated on July 21, 2025
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