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  • Review Article
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Eosinophils in vasculitis: characteristics and roles in pathogenesis

Nature Reviews Rheumatology volume 10, pages 474–483 (2014)Cite this article

Subjects

Key Points

  • Eosinophils are granulocytic cells that secrete a wide variety of cytokines, chemokines and other mediators that probably have direct and indirect roles in the pathogenesis of eosinophilic granulomatosis with polyangiitis (EGPA)

  • The clinical manifestations of eosinophil accumulation and activation include tissue fibrosis, thrombosis, and allergic inflammation but depend, to a large degree, on the particular tissue involved

  • Although data is scarce, eosinophils seem to have a central role in disease pathogenesis in all three clinical stages of EGPA

  • Clinical trials using novel therapeutic strategies that specifically target eosinophils are helping define the role of eosinophils in the pathogenesis of EGPA

Abstract

Eosinophils are multifunctional granular leukocytes that are implicated in the pathogenesis of a wide variety of disorders, including asthma, helminth infection, and rare hypereosinophilic syndromes. Although peripheral and tissue eosinophilia can be a feature of many types of small-vessel and medium-vessel vasculitis, the role of eosinophils has been best studied in eosinophilic granulomatosis with polyangiitis (EGPA), where eosinophils are a characteristic finding in all three clinical stages of the disorder. Whereas numerous studies have demonstrated an association between the presence of eosinophils and markers of eosinophil activation in the blood and tissues of patients with EGPA, the precise role of eosinophils in disease pathogenesis has been difficult to ascertain owing to the complexity of the disease process. In this regard, results of clinical trials using novel agents that specifically target eosinophils are providing the first direct evidence of a central role of eosinophils in EGPA. This Review focuses on the aspects of eosinophil biology most relevant to the pathogenesis of vasculitis and provides an update of current knowledge regarding the role of eosinophils in EGPA and other vasculitides.

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Figure 1: Characteristics of eosinophils.
Figure 2: Schematic representation of eosinophil trafficking.
Figure 3: Histopathology of EGPA.

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Acknowledgements

This work was supported in whole or in part by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, NIH. The authors thank C. R. Lee for assistance with the preparation of Figure 3.

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Authors and Affiliations

  1. Laboratory of Parasitic Diseases, National Institutes of Allergic and Infectious Diseases, National Institutes of Health, 9000 Rockville Pike, Bethesda, 20892, MD, USA

    Paneez Khoury & Amy D. Klion

  2. Vasculitis Translational Research Program, National Institutes of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, 9000 Rockville Pike, Bethesda, 20892, MD, USA

    Peter C. Grayson

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  1. Paneez Khoury
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All authors researched data for article, made a substantial contribution to discussion of content, wrote the article and reviewed/edited the manuscript before submission.

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Correspondence to Paneez Khoury.

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Khoury, P., Grayson, P. & Klion, A. Eosinophils in vasculitis: characteristics and roles in pathogenesis. Nat Rev Rheumatol 10, 474–483 (2014). https://doi.org/10.1038/nrrheum.2014.98

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