Eosinophilic granulomatosis with polyangiitis: Difference between revisions
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==Background== | ==Background== | ||
*Formerly known as Churg-Strauss | *Formerly known as Churg-Strauss Syndrome | ||
*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 | ||
{{Primary Vasculitis DDX}} | |||
==Clinical Features== | ==Clinical Features== | ||
Latest revision as of 17:44, 2 August 2023
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
Vasculitis Syndrome Types
- Large vessel
- Takayasu arteritis
- Giant cell arteritis (temporal arteritis)
- Medium-vessel
- Kawasaki disease
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger's disease)
- Primary angiitis of the central nervous system
- Small-vessel
- Henoch-Schönlein purpura
- ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegner's)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
- Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
- Behçet's disease]
- Secondary vasculitides and other/miscellaneous
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with other rheumatic diseases (e.g. SLE)
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 corticosteroids
Disposition
- Refer to rheumatology