EGPA (Eosinophilic Granulomatous PolyAngiitis) Flashcards
EGPA is previously designated as?
• CHURG-STRAUSS SYNDROME
• ALLERGIC GRANULOMATOSIS & ANGIITIS
What are the 5 disease characteristics of EGPA?
- VASCULITIS
- ASTHMA
- ALLERGIC RHINITIS
- ORGAN INFILTRATION BY EOSINOPHILS
- PERIPHERAL EOSINOPHILIA
What are the 6 clinical criteria used in the diagnosis of EGPA?
- ASTHMA
- PARANASAL ABNORMALITIES
- PULMONARY INFILTRATES (transient)
- EXTRAVASCULAR EOSINOPHILIC INFILTRATES
- > 10% PERIPHERAL BLOOD EOSINOPHILIA
- NEUROPATHY
What are the 3 combined clinical and histopathologic criteria used in the diagnosis of EGPA?
- eosinophil-rich granulomatous inflammation
- necrotizing vasculitis with eosinophilia and asthma
- positive ANCA test
What is the gender predominance in EGPA?
NONE
What is the mean age at diagnosis of EGPA?
40 years
What are the 4 histologic findings suggestive of EGPA in any organ?
- EOSINOPHILIC INFILTRATES
- EOSINOPHILIC GIANT CELL VASCULITIS OF SMALL ARTERIES & VEINS
- AREAS OF NECROSIS
- INTERSTITIAL & PERIVASCULAR GRANULOMAS
What are the 3 phases in the course of EGPA?
- ALLERGIC
- EOSINOPHILIC
- VASCULITIC
What are the 5 factor scoring system for the prediction of outcome in EGPA?
- AGE >65
- CARDIAC INVOLVEMENT
- GI INVOLVEMENT
- RENAL INSUFFICIENCY
- ENT INVOLVEMENT (ABSENCE)
What is the primary treatment for EGPA?
CORTICOSTEROIDS
What are the 12 immunosuppressive therapy used together with corticosteroids in the treatment of EGPA?
- CYCLOPHOSPHAMIDE
- AZATHIOPRINE
- MMF
- METHOTREXATE
- LEFLUNOMIDE
- IFN-α
- TNF BLOCKING AGENTS
- IVIG
- PLASMA EXCHANGE
- RITUXIMAB
- OMALIZUMAB
- MEPOLIZUMAB
What immunosuppressive therapy was shown to be effective for EGPA in refractory and relapsing cases?
RITUXIMAB
What is a monoclonal antibody directed against the IgE receptor which has been used in steroid-resistant patients?
OMALIZUMAB
This is associated with a worse prognosis in EGPA?
A shorter duration of asthma prior to the onset of vasculitis
