Rheumatology

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Churg–Strauss angiitis
Best Practice & Research: Clinical Rheumatology, 06/30/09

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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