A multisystem disease characterized by necrotizing granulomatous vasculitis with eosinophilia that most commonly involves the lungs and the skin; can also affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems
Epidemiology
Etiology
Pathophysiology
Clinical features
- Prodromal phase
- Severe allergic asthma attacks (chief concern)
- Allergic rhinitis/sinusitis
- Eosinophilic phase
- Lung disease
- Pericarditis
- Gastrointestinal involvement: bleeding, ischemia, perforation
- Vasculitic phase
- Skin nodules, palpable purpura
- Mononeuritis multiplex (loss of motor and sensory function with wrist or foot drop), symmetric or asymmetric polyneuropathy
- Pauci-immune glomerulonephritis
Diagnostics
- CBC: peripheral blood eosinophilia (> 1500 cells/mcL or > 10% of the leukocyte count)
- ANCA present in ∼ 40%: of those, MPO-ANCA in 74–90%
- ↑ IgE and ↑ IgG4 levels