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:

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 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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