Treatment of refractory Churg-Strauss-Syndrome (CSS) by TNF-α blockade

Churg-Strauss-Syndrome (CSS) often takes a mild course and is in many cases treated successfully by glucocorticosteroids (GC) alone. However, there are also several reports demonstrating the necessity of more intensive treatment in life threatening courses with cyclophosphamide and in less severe cases with other immunosuppressive or immunomodulatory drugs like azathioprine, methotrexate or interferon alpha. Relapses of the CSS are detected clinically and serologically and may require cyclophosphamide therapy as well as high-dose GC. We treated 3 cases between 2000 and 2001 that not only experienced a severe relapse (of the heart and the central nervous system) but also proved to be refractory to cyclophosphamide and GC therapy. In the absence of other options we decided to apply TNF-alpha blockers (etanercept in one case and remicade in the two other). This experimental treatment proved to be effective and safe and induced complete remission in one patient and partial remission in the second and at least stopped disease progression in the third. The BVAS 1 markedly improved after additional treatment with TNF-alpha blockers.