🌟 A systemic vasculitis where asthma and pulmonary infiltrates precede eosinophilia, sinusitis, vasculitis, neuropathy, and sometimes a restrictive cardiomyopathy.

📖 About

Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly Churg-Strauss Syndrome, is a small-to-medium vessel necrotizing vasculitis with eosinophil-rich granulomatous inflammation.
Typically evolves in 3 phases:
- 🔹 Allergic phase – asthma, rhinitis, sinusitis.
- 🔹 Eosinophilic phase – eosinophilic pneumonia, GI involvement.
- 🔹 Vasculitic phase – systemic small-vessel vasculitis with multi-organ damage.

🩺 Clinical Features

🌬️ Respiratory: Asthma, allergic rhinitis, transient pulmonary infiltrates, cough, dyspnoea.
🩸 Skin: Palpable purpura, nodules, ulcers.
⚡ Neurology: Peripheral neuropathy, especially mononeuritis multiplex.
❤️ Cardiac: Restrictive cardiomyopathy, myocarditis, arrhythmias → leading cause of mortality.
🧑‍⚕️ Renal: GN with proteinuria/haematuria (less severe than GPA).
🌡️ Systemic: Fever, weight loss, malaise.

✅ Churg-Strauss (ACR) Diagnostic Criteria

≥4 of 6 features → sensitivity 85%, specificity 99%:

Asthma
Eosinophilia >10% of WCC
Neuropathy (mono/polyneuritis multiplex)
Transient pulmonary infiltrates
Sinus disease
Extravascular eosinophilia on biopsy

🔗 Associations

Montelukast (anti-leukotriene therapy) may be linked in some cases.
Overlap with eosinophilic pneumonia & chronic eosinophilic bronchitis.

🧪 Investigations

📊 Bloods: Eosinophilia (>1.5 × 10⁹/L), raised ESR/CRP, check renal function.
🧬 ANCA: MPO+ (p-ANCA) ~50%, PR3+ (c-ANCA) ~40%.
🩻 Imaging: CXR/CT → transient infiltrates, ground-glass opacities, nodules.
💨 Lung function: Asthma pattern ± restrictive in late disease.
⚡ Nerve conduction: Confirms neuropathy.
🔬 Biopsy: Necrotizing granulomas with eosinophilic infiltrates.

💊 Management

Specialist rheumatology/immunology input is essential.
🔥 Corticosteroids: High-dose prednisolone first-line.
💉 Immunosuppressants:
- Cyclophosphamide for severe/organ-threatening disease (renal, cardiac, neuro).
- Azathioprine or Methotrexate as steroid-sparing for maintenance.
🧬 Biologics: Mepolizumab (anti-IL-5) effective in refractory eosinophilic disease.
📋 Supportive: Manage asthma (ICS/LABA), treat cardiac complications, monitor steroid toxicity.

📉 Prognosis

Variable – depends on organ involvement and response to therapy.
❤️ Cardiac involvement → major determinant of mortality.
Early diagnosis + immunosuppression → improves long-term survival.

📚 References

3 Clinical Cases - Eosinophilic Granulomatosis with Polyangiitis (EGPA / Churg–Strauss) 🧪🫁

Case 1 - Asthma with eosinophilia 🫁: A 42-year-old woman with long-standing asthma and allergic rhinitis develops worsening wheeze, sinusitis, and marked peripheral eosinophilia (3.5 × 10⁹/L). CXR: transient pulmonary infiltrates. Teaching: EGPA often begins with asthma and allergic features. Eosinophilia plus fleeting lung infiltrates are a red flag. Steroids are first-line treatment and may induce remission.
Case 2 - Vasculitic neuropathy ⚡: A 55-year-old man with severe adult-onset asthma presents with painful foot drop and paraesthesia. Nerve conduction studies show mononeuritis multiplex. Bloods: eosinophilia, p-ANCA positive. Teaching: EGPA is a small-vessel vasculitis. Neuropathy is common, due to vasculitic infarction of vasa nervorum. Management requires high-dose corticosteroids ± immunosuppressants (e.g. cyclophosphamide, rituximab).
Case 3 - Cardiac involvement ❤️: A 48-year-old woman with asthma and arthralgia presents with chest pain, palpitations, and breathlessness. Echo: LV dysfunction, myocarditis. Troponin mildly elevated. Teaching: Cardiac disease (myocarditis, pericarditis, coronary vasculitis) is a major cause of mortality in EGPA. Early recognition is vital. Treatment = corticosteroids plus immunosuppression; cardiac monitoring essential.

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Eosinophilic granulomatosis (Churg Strauss)