Churg Strauss syndrome

Churg Strauss is a rare, small-vessel vasculitis of unknown aetiology  

The triad for diagnosis is: 

• Asthma 
• Eosinophilia 
• Vasculitis of 2 or more extrapulmonary organs 

The asthma is often late onset.

The vasculitis of Churg Strauss

• Most commonly affects the lungs 
• Causes mononeuritis multiplex in up to 75% of patients 
• Affects the skin (causing vasculitic purpura, nodules or livedo reticularis) in around 60% 
• Glomerulonephritis (focal and segmental) occurs in up to 47% of patients but renal failure is rare 

Associations of Churg Strauss include 

• Nasal polyps 
• Sinusitis 
• Allergic rhinitis 
• Abdominal pain 

ANCA is positive in around a third of patients, most commonly pANCA 

Treatment is with high-dose steroids. 

References:

Conron, M. and Beynon, H. Churg-Strauss syndrome. Thorax. 2000; 55(10): 870-877. Eustace, J., Nadasdy, T. and Choi, M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. 1999; 10(9): 2048-2055.