Churg-Strauss syndrome: Difference between revisions

(Churg-Strauss syndrome)
 
 
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==Background==
#REDIRECT[[Eosinophilic granulomatosis with polyangiitis]]
*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
 
==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
 
==See Also==
 
==External Links==
 
==References==
<UpToDate>

Latest revision as of 03:37, 13 November 2016