Churg-Strauss syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Eosinophilic granulomatosis with polyangiitis (EGPA) | *Eosinophilic granulomatosis with polyangiitis (EGPA) | ||
*Chronic sinusitis, asthma, and peripheral eosinophilia | *Chronic [[sinusitis]], [[asthma]], and peripheral [[eosinophilia]] | ||
*Vasculitis of small and medium sized vessels | *Vasculitis of small and medium sized vessels | ||
*Lung and skin are most commonly involved | *Lung and skin are most commonly involved | ||
Revision as of 05:08, 5 October 2016
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
Evaluation
- Typically a clinical diagnosis
- Labs with elevated eosinophils, ESR, CRP, ANCA
- CXR with transient, patchy opacities
Management
Initiation Therapy
- Prednisone 0.5-1.5mg/kg/day
- Cyclophosphamide
Maintenance Therapy
- Azathioprine
- Methotrexate
- Leflunomide
- Inhaled glucocorticoids
Disposition
- Refer to rheumatology