Clinical manifestations and treatment of Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis affecting small to medium-sized vessels, and characterized by asthma, blood hypereosinophilia, and eosinophil-rich granulomatous inflammation of the respiratory tract. In the past few years the pathogenesis of the disease and its clinical manifestations have been clarified, fostering important advances in the treatment of CSS. Systemic corticosteroids are still considered the cornerstone of treatment. Many issues need to be addressed, such as how to maintain remission, prevent disease relapses, and treat refractory disease. This review provides a clinical overview of CSS and a summary of the current treatments and novel therapies.