Rheumatology Advance Access originally published online on April 8, 2008
Rheumatology 2008 47(6):804-808; doi:10.1093/rheumatology/ken033
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Eotaxin-3 is involved in Churg–Strauss syndrome – a serum marker closely correlating with disease activity
1Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 2Department of Internal Medicine 3, Medical University of Vienna, Vienna, Austria, 3Department of Internal Medicine 3, University of Jena, Jena, Germany, 4Department of Neuropathology, 5Department of Pathology, Medical University of Vienna, Vienna, Austria and 6Deparment of Rheumatology, University of Schleswig-Holstein, Kiel, Germany.
Correspondence to: J. Zwerina, Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Krankenhausstrasse 12, 91054 Erlangen, Germany. E-mail: jochen.zwerina{at}uk-erlangen.de
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Abstract |
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Objective. Churg–Strauss Syndrome (CSS) is characterized by excessive eosinophil accumulation in peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. The contribution of eosinophil-chemotactic cytokines (eotaxin family) to eosinophilia and disease activity in CSS is unknown. Thus, we compared serum levels of the eotaxin family members in CSS patients with healthy and disease controls.
Methods. Forty patients with CSS diagnosed according to ACR 1990 criteria, 30 healthy controls (HC) and 57 disease controls (28 asthma, 20 small vessel vasculitis, 9 hypereosinophilic syndrome) were studied. Clinical data were collected and serum levels of eotaxin-1, -2 and -3 were determined by ELISA. Further, immunohistochemistry was applied to identify eotaxin-3 expression in tissue biopsies from patients with CSS.
Results. In contrast to eotaxin-1 and -2, eotaxin-3 was highly elevated in serum samples of active CSS patients and correlated highly significantly with eosinophil counts, total immunoglobulin E (IgE) levels and acute-phase parameters. Moreover, eotaxin-3 was not elevated in other eosinophilic and vasculitic diseases. Immunohistochemical analysis revealed strong expression of eotaxin-3 in endothelial and inflammatory cells in affected tissues of active CSS patients.
Conclusions. This study reveals the specific association of elevated eotaxin-3 expression with high disease activity and eosinophilia in CSS patients. Eotaxin-3 might thus be a pathogenic player, biomarker and potential therapeutic target in CSS.
KEY WORDS: Churg–Strauss syndrome, Vasculitis, Eosinophilia, Eotaxins
Submitted 5 July 2007;
revised version accepted 14 January 2008.
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