Churg-Strauss syndrome

Background

• Eosinophilic granulomatosis with polyangiitis (EGPA)
• 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

• Prodrome
  • Atopic diseases, allergic rhinitis, and asthma

• Eosinophilic phase
  • Multiorgan involvement, particularly lung and GI tract
  • Caused by eosinophilic infiltration into these organs

• 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 vasculitides

Diagnosis

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

Management

• Initiation Therapy
  • Prednisone 0.5-1.5 mg/kg/day
  • Cyclophosphamide

• Maintenance Therapy
  • Azathioprine
  • Methotrexate
  • Leflunomide
  • Inhaled glucocorticoids

Disposition

• Refer to rheumatology

References

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