Eosinophilic granulomatosis with polyangiitis

Background

  • Formerly known as Churg-Strauss syndrome
  • Chronic sinusitis, asthma, and peripheral eosinophilia
  • Vasculitis of small and medium sized vessels
  • Lung and skin are most commonly involved

Clinical Features

Classically develops in three sequential phases:

  1. Prodrome
    • Atopic diseases, allergic rhinitis, and asthma
  2. Eosinophilic phase
    • Multiorgan involvement, particularly lung and GI tract
    • Caused by eosinophilic infiltration into these organs
  3. Vasculitic phase
    • Life threatening vasculitis associated with vascular and extravascular granulomatosis
    • May present with nonspecific signs such as fever, weight loss, malaise

Differential Diagnosis

  • Aspirin-exacerbated respiratory disease
  • Chronic eosinophilic pneumonia
  • Allergic bronchopulmonary aspergillosis
  • Hypereosinophilic syndrome
  • Other vasculitis syndromes

Evaluation

  • Typically a clinical diagnosis
  • Labs with elevated eosinophils, ESR, CRP, ANCA
  • CXR with transient, patchy opacities

Management

Acute Therapy

Maintenance Therapy

Disposition

  • Refer to rheumatology

See Also

External Links

References