Eosinophilic granulomatosis with polyangiitis: Difference between revisions
ClaireLewis (talk | contribs) (Created page with "==Background== *Formerly known as Churg-Strauss syndrome *Chronic sinusitis, asthma, and peripheral eosinophilia *Vasculitis of small and medium sized vessels *Lun...") |
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==Management== | ==Management== | ||
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*[[Prednisone]] 0.5-1.5mg/kg/day | *[[Prednisone]] 0.5-1.5mg/kg/day | ||
*[[Cyclophosphamide]] | *[[Cyclophosphamide]] | ||
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==References== | ==References== | ||
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[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
Revision as of 03:34, 30 December 2016
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:
- 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 vasculitis syndromes
Evaluation
- Typically a clinical diagnosis
- Labs with elevated eosinophils, ESR, CRP, ANCA
- CXR with transient, patchy opacities
Management
Acute Therapy
- Prednisone 0.5-1.5mg/kg/day
- Cyclophosphamide
Maintenance Therapy
- Azathioprine
- Methotrexate
- Leflunomide
- Inhaled glucocorticoids
Disposition
- Refer to rheumatology