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AND THE ANSWERS ARE . . .

  1. Asthma is an infrequent manifestation in individuals with CSS
    False
  2. Pulmonary infiltrates occur in a large percentage of those with the CSS
    True
  3. Eosinophil accumulation is a major cause of tissue damage in the CSS
    True
  4. Renal involvement in the CSS primarily involves inflammation of the local mid-sized arteries
    False
  5. The prognosis for survival in treated CSS is very good.
    True

Discussion
Almost all individuals diagnosed with the CSS have a history of asthma, currently or in the recent past. Allergic rhinitis and/or nasal polyposis are also common in such individuals. However, unlike the situation in uncomplicated asthma (where the chest x-ray is normal except for hyper-inflation), pulmonary infiltrates occur in a sizable percentage of CSS cases. These infiltrates are often due to the presence of eosinophil-rich granulomas generally extracellular. There is often eosinophilia in the blood and other organs where they likely damage the tissue by the effects of released eosinophil cationic proteins.

A small vessel vasculitis involving both the arteries and veins is common in CSS. Associated with this is a segmental glomerulonephritis in about 40% of cases. Here may be small vessel vasculitis but, unlike polyarteritis nodosa, the mid-sized renal arteries s are not commonly involved. Anti-neutrophil cytoplasmic antibodies (ANCA) are found in about 60% of CSS, generally associated with the presence of vasculitis. This ANCA is usually of the perinuclear type, directed against myeloperoxidase. Recent studies suggest that different patterns of organ involvement may occur in those CSS cases in which the ANCA is positive vs negative

When treated appropriately with corticosteroids (and cytotoxic drugs when needed), the prognosis in CSS is very good (80% survival at 5 years) though relapses are common when drug treatment is withdrawn.

References
Curr Opin Rheumatol 2007;19:25-32
Semin Respir Crit Care Med. 2006 ;27:148-57
Curr Opin Nephrol Hypertens. 2003 ;12: 267-72

 



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