EGPA Flashcards
(25 cards)
What is Eosinophilic Granulomatosis with Polyangiitis (EGPA)?
EGPA is a rare, ANCA-associated necrotizing vasculitis affecting small to medium vessels, characterized by asthma, eosinophilia, and granulomatous inflammation.
Which previous name was used for EGPA?
Churg–Strauss syndrome.
What is the typical age of onset for EGPA?
Middle-aged adults (peak between 40–60 years).
What are the three classic phases of EGPA?
- Allergic phase – asthma, allergic rhinitis
- Eosinophilic phase – eosinophilic organ infiltration
- Vasculitic phase – systemic vasculitis with multi-organ involvement
Which respiratory condition is almost always present in EGPA?
Asthma (present in >90% of cases).
Name 3 respiratory features of EGPA.
Asthma, sinusitis, transient pulmonary infiltrates.
What are 2 common neurological manifestations of EGPA?
Mononeuritis multiplex (e.g., foot drop), peripheral neuropathy.
What skin findings are seen in EGPA?
Palpable purpura, subcutaneous nodules, urticaria.
What GI symptoms may occur in EGPA?
Abdominal pain, GI bleeding due to eosinophilic gastroenteritis or vasculitis.
What cardiac complications may occur?
Myocarditis, pericarditis, heart failure; major cause of mortality.
What is the key hematological abnormality in EGPA?
Eosinophilia >10% or >1.5 × 10⁹/L.
What differentiates EGPA from GPA and MPA?
EGPA has asthma, eosinophilia, and granulomas. GPA has upper airway + renal + cavitating lung lesions. MPA is usually renal + pulmonary capillaritis without granulomas.
Which antibody is commonly associated with EGPA?
p-ANCA (MPO-ANCA) – positive in ~40–60% of patients.
What is seen on lung imaging in EGPA?
Transient, patchy, non-fixed pulmonary infiltrates.
What does biopsy typically show in EGPA?
Eosinophilic necrotizing granulomatous inflammation with vasculitis.
What blood tests are relevant in suspected EGPA?
• CBC (eosinophilia)
• ANCA (MPO)
• CRP/ESR (raised)
• IgE (elevated)
• Renal function, LFTs
What is the role of ANCA in EGPA diagnosis?
MPO-ANCA may support diagnosis but is not always positive; clinical features are key.
What cardiac investigations are indicated?
ECG, echocardiogram, and cardiac MRI (if myocarditis suspected).
What is the first-line treatment for mild EGPA?
Oral corticosteroids (e.g., prednisolone 1 mg/kg/day).
When are immunosuppressive agents indicated?
In organ-threatening or life-threatening disease – add cyclophosphamide or rituximab.
What agent is used for steroid-sparing maintenance?
Azathioprine, methotrexate, or mycophenolate mofetil.
Which biologic is used in refractory EGPA?
Mepolizumab (anti–IL-5) – useful especially for asthma and eosinophilic inflammation.
How is cardiac involvement managed in EGPA?
Aggressive immunosuppression (steroids + cyclophosphamide), cardiac monitoring, and heart failure management if needed.
What is the main cause of death in EGPA?
Cardiac involvement (e.g., myocarditis, heart failure, arrhythmia).
