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ANCA-Associated Vasculitis Pathogenesis: A Commentary

  • Vasculitis (L Espinoza, Section Editor)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

The ANCA-associated vasculitides are a group of small vessel vasculitides characterized by autoantibodies recognizing the neutrophil cytoplasmic antigens PR3 and MPO. We examine the current clinical and molecular immunology understanding of ANCA-associated vasculitides and discuss the current needs in our understanding of the pathogenic mechanisms of these rare diseases.

Recent Findings

The majority of efforts to understand the pathogenesis of these diseases have focused on dissecting neutrophil biology because the neutrophil is the primary expressor of ANCA autoantigens. However, a number of important genetic, clinical, and cellular biology observations suggest that attempts to understand the pathogenesis of ANCA vasculitides should move away from emphasis on the role of the neutrophil and instead re-focus on the potential role of other immune cell mediators.

Summary

Whether or not neutrophils are the key determinant of ANCA-associated vasculitis pathogenesis should be revisited in detail. A neutrophil-centric view of the pathogenesis of these diseases cannot fully account for important genetic, clinical, and cellular biology observations that implicate important and under-appreciated roles for monocytes and T cells. Refocusing on these findings will likely lead to new discovery of novel therapeutic targets and the identification of clinically useful biomarkers for disease activity.

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Acknowledgements

The work reported in this publication was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health under Award Number T32AR048522 and by National Institutes of Health Award Number RO1 DE 12354-15A1. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Dr. Gapud is supported by funding from the National Institutes of Health Award Number T32AR048522. Dr. Antiochos is supported by the Jerome L. Greene Foundation and National Institutes of Health Award Number RO1 DE 12354-15A1. Dr. Seo was supported by the Dr. Darwin James Liao Discovery Fund.

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  1. Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, MFL Center Tower, Ste. 5300, Baltimore, MD, 21224, USA

    Eric J. Gapud, Philip Seo & Brendan Antiochos

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  1. Eric J. Gapud
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  2. Philip Seo
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Correspondence to Brendan Antiochos.

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Gapud, E.J., Seo, P. & Antiochos, B. ANCA-Associated Vasculitis Pathogenesis: A Commentary. Curr Rheumatol Rep 19, 15 (2017). https://doi.org/10.1007/s11926-017-0641-0

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