ILD
Churg-Strauss Syndrome
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Churg-Strauss Syndrome
, Churg-Strauss, Allergic Granulomatosis
Epidemiology
Rare disease seen most often in men
Pathophysiology
Similar to Classic
Polyarteritis Nodosa
Small Vessel,
Granuloma
tous
Vasculitis
Multiple organ system involvement
Interstitial Lung Disease
Phases
Phase 1: Upper Respiratory
Severe Asthma
Allergic Rhinitis
Phase 2: Lower Respiratory
Eosinophil
ic lung infiltrate
Phase 3:
Vasculitis
(occurs within 3 years of onset)
Granuloma
tous
ANCA
-associated
Small Vessel Vasculitis
Signs
Interstitial Lung Disease
Eosinophil
ic tissue infiltration
Labs
Antineutrophil Cytoplasmic Antibody
(
ANCA
)
Positive in 70% of cases (usually
MPO-ANCA
)
Complete Blood Count
(CBC)
Eosinophilia
>10%
Complications (often cause of death)
Coronary arteritis
Myocarditis
Differential Diagnosis
Polyarteritis Nodosa
(medium vessel
Vasculitis
)
ANCA
-Associated
Small Vessel Vasculitis
Granulomatosis with Polyangiitis
(previously known as
Wegener's Granulomatosis
)
Microscopic Polyangiitis
Management
High-dose
Corticosteroid
s
Control vascular injury
Hypertension
Diabetes Mellitus
Tobacco Abuse
Hyperlipidemia
References
Calabrese in Ruddy (2001) Kelly's Rheum, p. 1179-81
Eustace (1999) J Am Soc Nephrol 10(9):2048-55 [PubMed]
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