Churg-Strauss syndrome

- eosinophilic granulomatous inflammation of respiratory tract with small and medium vessels necrotizing vasculitis
- pANCA is positive (50%)
- leukotriene inhibitor allow steroid doses to be lowered and this may unmask underlying vasculitis
- causing focal segmental glomerulonephritis
- principle cause of morbidity and mortality are myocarditis and myocardial infarction secondary to coronary arteritis
- serum IgE is commonly elevated and correlates with disease severity
- treatment: glucocorticoids, azathioprine, cyclophosphamide
- with treatment, 1 year survival is 90% and 5 year survival is 62%

mnemonic : BEAN SAP


BE: Blood Eosinophilia
A  : Asthma
N  : Neuropathy (mononeuritis multiplex) - usually common peroneal nerve
S  :  Sinus abnormality
A  : Allergies
P  : Perivascular eosinophils / vasculitis