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Churg-Strauss Syndrome Pulmonary Manifestations (EGPA)

Pulmonary involvement in eosinophilic granulomatosis with polyangiitis: severe asthma, eosinophilic pneumonia, and small-vessel vasculitis with bronchospasm and pulmonary infiltrates.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Churg-Strauss Syndrome Pulmonary Manifestations (EGPA)?

EGPA pulmonary disease begins with severe, often steroid-dependent asthma typically preceding vasculitis by 8-10 years. Asthma is poorly controlled, requires high-dose inhaled corticosteroids and oral steroid courses, and may worsen rather than improve with leukotriene modifiers (controversial association with EGPA induction).

Eosinophilic phase: peripheral eosinophilia >1500/μL or >10%; transient migratory pulmonary infiltrates (Löffler-like); eosinophilic pneumonia (chronic or acute); pleural effusion (eosinophilic); pulmonary nodules. CT shows ground-glass opacities, consolidation, centrilobular nodules, septal thickening.

Vasculitic phase: small-vessel vasculitis with palpable purpura, mononeuritis multiplex (vasculitic peripheral neuropathy), cardiac involvement (eosinophilic myocarditis, coronary vasculitis — leading cause of mortality), GI involvement (eosinophilic enteritis, mesenteric vasculitis), renal involvement (less severe than other ANCA vasculitides), CNS (rare). Granulomatous extravascular eosinophilic infiltrates pathognomonic on biopsy.

Symptoms

Severe, late-onset, steroid-dependent asthma (cardinal feature)
Allergic rhinitis and chronic sinusitis with nasal polyps
Migratory pulmonary infiltrates
Cough, wheeze, dyspnea worsening despite asthma therapy
Hemoptysis (alveolar hemorrhage)
Mononeuritis multiplex (foot drop, wrist drop, asymmetric weakness)
Palpable purpura, skin nodules, livedo reticularis
Cardiac symptoms (chest pain, heart failure — myocarditis)
Gastrointestinal pain, bleeding (mesenteric vasculitis)
Constitutional symptoms (fever, weight loss, malaise)

Risk Factors

Pre-existing severe asthma with eosinophilia
Allergic rhinitis, nasal polyps, sinusitis
Mean age 40-50 years (rare in children)
Slight female predominance
Genetic factors (HLA-DRB1*04, IL-10 polymorphisms)
Some studies suggest leukotriene receptor antagonists may unmask EGPA (controversial — likely steroid taper effect)
Possible association with biologic asthma therapy initiation (omalizumab, mepolizumab unmasking)

When to See a Doctor?

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

  • Worsening or refractory adult-onset asthma with peripheral eosinophilia
  • New peripheral neuropathy (foot/wrist drop) in asthma patient
  • Migratory pulmonary infiltrates in asthma patient
  • Palpable purpura with constitutional symptoms
  • Heart failure or chest pain in young patient with asthma history
  • Pulmonology, rheumatology, and cardiology multidisciplinary urgent referral

Treatment Methods

01
Diagnostic workup: CBC with peripheral eosinophil count (>1500/μL or >10%), ANCA testing (40% MPO-ANCA positive), CT chest/sinus, echocardiography and cardiac MRI, nerve conduction studies for neuropathy, biopsy of affected tissue (skin, lung, kidney, nerve)
02
Biopsy findings: small-vessel vasculitis, extravascular eosinophilic granulomas, perivascular eosinophilic infiltrate; pulmonary biopsy may show eosinophilic pneumonia
03
Five Factor Score (FFS): predicts severity (cardiac, GI, CNS, renal involvement, age >65); FFS=0 → corticosteroids alone; FFS≥1 → combination therapy
04
Induction therapy mild-moderate (FFS=0): prednisolone 1 mg/kg/day; rituximab as steroid-sparing alternative; azathioprine or methotrexate maintenance
05
Induction therapy severe (FFS≥1, alveolar hemorrhage, severe cardiac involvement): high-dose corticosteroids + cyclophosphamide IV pulses (15 mg/kg every 2-4 weeks for 3-6 months) OR rituximab 375 mg/m² weekly × 4
06
Mepolizumab (anti-IL-5) 300 mg SC monthly (FDA-approved 2017 for EGPA): improves remission rate, reduces relapses, decreases steroid burden; first targeted therapy for EGPA
07
Benralizumab (anti-IL-5Rα): emerging evidence in EGPA; depletes eosinophils via ADCC
08
Maintenance therapy: azathioprine, methotrexate, or mycophenolate; mepolizumab for refractory or relapsing; lower-dose prednisolone 5-10 mg
09
Asthma control: high-dose ICS, LABA, leukotriene modifiers (caution but generally continued), biologics (mepolizumab dual indication)
10
Cardiac involvement: aggressive immunosuppression (cyclophosphamide or rituximab), heart failure management, anticoagulation if mural thrombus, ICD if arrhythmia risk
11
Surgical interventions: rare; nasal polyp resection for refractory sinus disease, cardiac transplant in end-stage cardiomyopathy
12
Prognosis: overall 5-year survival 80-90%; cardiac involvement major predictor of mortality; ANCA-positive patients have more vasculitis features and renal involvement; ANCA-negative more eosinophilic and cardiac involvement
13
Long-term monitoring: relapses common (30-50%), monitor with peripheral eosinophilia, ANCA, organ function; surveillance imaging; cardiology follow-up

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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.