Eosinophilic granulomatosis with polyangiitis: Difference between revisions
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Classically develops in three sequential phases: | Classically develops in three sequential phases: | ||
#Prodrome | #Prodrome | ||
#*Atopic diseases, allergic rhinitis, and asthma | #*Atopic diseases, [[allergic rhinitis]], and [[asthma]] | ||
#Eosinophilic phase | #Eosinophilic phase | ||
#*Multiorgan involvement, particularly lung and GI tract | #*Multiorgan involvement, particularly lung and GI tract | ||
#*Caused by eosinophilic infiltration into these organs | #*Caused by eosinophilic infiltration into these organs | ||
#Vasculitic phase | #Vasculitic phase | ||
#*Life threatening vasculitis associated with vascular and extravascular granulomatosis | #*Life threatening [[vasculitis]] associated with vascular and extravascular granulomatosis | ||
#*May present with nonspecific signs such as fever, weight loss, malaise | #*May present with nonspecific signs such as [[fever]], weight loss, malaise | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Aspirin-exacerbated respiratory disease | *[[Aspirin]]-exacerbated respiratory disease | ||
*Chronic eosinophilic pneumonia | *Chronic eosinophilic pneumonia | ||
*Allergic bronchopulmonary aspergillosis | *Allergic bronchopulmonary [[aspergillosis]] | ||
*Hypereosinophilic syndrome | *Hypereosinophilic syndrome | ||
*Other [[vasculitis syndromes]] | *Other [[vasculitis syndromes]] | ||
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*[[Methotrexate]] | *[[Methotrexate]] | ||
*Leflunomide | *Leflunomide | ||
*Inhaled | *[[Inhaled corticosteroids]] | ||
==Disposition== | ==Disposition== | ||
Revision as of 23:38, 17 October 2019
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 corticosteroids
Disposition
- Refer to rheumatology