Churg-Strauss-Syndrom (CSS) is a disorder characterized by pulmonary and systemic small-vessel vasculitis, extravascular granuloma and hypereosinophilia that occurs in patients with asthma and allergic rhinitis. The involvement of the central nervous system (CNS) is less common than peripheral neuropathy, but causes significant morbidity and mortality in affected patients.
Systemic glucocorticoids in combination with cyclophosphamide represent the treatment of choice in patients with systemic involvement of heart, nervous system or kidney. In case of intolerance or failure to cyclophosphamide, plasma exchange, interferon alpha, or other immunosuppressants such as mycophenolat mofetil have been suggested as alternative treatment options.
Recently, the successful use of rituximab (RTX) in patients with ANCA-associated vasculitis as an anti-CD20 directed B-cell depleting antibody was reported. However, only few case reports on the use of RTX in CSS are available, none of them including patients with CNS.