Makindo Medical Notes

🌟 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

• NCBI Bookshelf – EGPA
• American College of Rheumatology
• UpToDate – EGPA