Churg–Strauss angiitis
Sinico RA et al. – Churg–Strauss angiitis or syndrome (CSA) is defined as an eosinophil-rich and granulomatous inflammation involving the respiratory tract, coupled with necrotising vasculitis affecting small- to medium-sized vessels, and is associated with asthma and eosinophilia. This review article outlines the diagnosis and classification and treatment available for this disease.- It is usually classified among the ANCA-associated systemic vasculitides (AASVs)
- However, ANCA status was shown to segregate with clinical phenotype in two recent studies
- ANCA-positive pts were more likely to have disease manifestations associated with small-vessel vasculitis
- Whereas ANCA-negative cases were more likely to have cardiac and lung involvement
- Preliminary results suggest that ANCA-positive and ANCA-negative pts also might have a different genetic background
- Corticosteroids remain the initial treatment of CSA
- Addition of cyclophosphamide is indicated in treatment of pts with poor-prognosis factors or in pts w/o poor-prognosis factors but those that are prone to relapses
- Length of the maintenance therapy remains to be established
- However, majority of pts require long-term corticosteroids treatment to control asthma
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