Diagnostic and therapeutic management of Churg-Strauss syndrome.
Churg-Strauss syndrome is a rare small-vessel vasculitis that is associated with asthma, granulomatous inflammation, peripheral/tissue eosinophilia and a positive antineutrophil cytoplasmic antibody status (in approximately 40% of patients). The disease can be organ- and life-threatening, either due to tissue eosinophil infiltration such as myocarditis or due to vasculitis manifestations, for example glomerulonephritis. Furthermore, life-threatening disease can also occur due to the side effects of immunosuppression, for example, infection. A thorough diagnostic work-up should be performed in order to identify all organs involved and to rule out other disorders with similar features, such as hypereosinophilic syndrome. Therapeutic management is conducted according to disease stage and activity. Glucocorticoids remain the mainstay of therapy; however, further immunosuppressants (e.g., cyclophosphamide for life-threatening disease) are usually required. Future promising therapy options target cytokines involved in the disease process, such as IL-5.
Holle
Julia U
JU
University Hospital Schleswig-Holstein, Campus Luebeck, Dept of Rheumatology and Klinikum Bad Bramstedt, Dept of Rheumatology and Immunology, Oskar-Alexander-Strasse 26, 24576 Bad, Bramstedt, Germany. julia.holle@uk-sh.de
Moosig
Frank
F
Gross
Wolfgang L
WL