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Cutting Edge Issues in the Churg–Strauss Syndrome

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Abstract

Churg–Strauss syndrome (CSS) is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.

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Acknowledgments

This work has been supported by grant from the Polish Ministry of Science and Higher Education—Iuventus Plus for year 2011.

We would like to acknowledge Michał Wodkowski and Stanislaw Polanski, for their help in manuscript editing.

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  1. Department of Medicine, Jagiellonian University Medical College, Skawińska 8, 31 - 066, Kraków, Poland

    Wojciech Szczeklik, Bogdan Jakieła & Jacek Musiał

  2. Department of Pathomorphology, Jagiellonian University Medical College, Kraków, Poland

    Dariusz Adamek

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  1. Wojciech Szczeklik
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Correspondence to Wojciech Szczeklik.

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Szczeklik, W., Jakieła, B., Adamek, D. et al. Cutting Edge Issues in the Churg–Strauss Syndrome. Clinic Rev Allerg Immunol 44, 39–50 (2013). https://doi.org/10.1007/s12016-011-8266-y

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