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IgA Vasculitis in Adults: a Rare yet Challenging Disease

  • Vasculitis (C Dejaco and C Duftner, Section Editors)
  • Published:
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Abstract

Purpose of Review

IgA vasculitis (IgAV) is a rare and poorly understood systemic vasculitis in adults. Its diagnosis and treatment remain a challenge. Herein, we review the clinical manifestations, diagnosis, management, and prognosis of IgAV in adults.

Recent Findings

The clinical course of IgAV in adults appears to be different from pediatric IgAV, especially due to its higher risk of evolving into end-stage renal disease. Rising awareness and interest in adult-onset IgA vasculitis has resulted in recent increasing number of publications on different treatment experiences. However, there is still controversy over the role of glucocorticoid (GC) and different immunosuppressive therapies such as cyclophosphamide, rituximab, and mychophenolate mofetil for more severe IgAV. Data regarding potential benefits of targeting the mucosal immune system, toll-like receptors, complements, and tyrosine kinase inhibitors in the treatment of IgA nephropathy are emerging.

Summary

High quality evidence or guidelines in the treatment of severe IgAV are lacking and there is still a great need for controlled trials.

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Authors and Affiliations

  1. Center for Vasculitis Care and Research, Cleveland Clinic, 9500 Euclid Avenue/A50, Cleveland, OH, 44195, USA

    Kinanah Yaseen

  2. Director of Medical Kidney Pathology, Department of Anatomic Pathology, Cleveland Clinic, 9500 Euclid Ave/L25, Cleveland, OH, 44195, USA

    Leal C Herlitz

  3. Center for Vasculitis Care and Research, Cleveland Clinic, 9500 Euclid Avenue/A50, Cleveland, OH, 44195, USA

    Alexandra Villa-Forte

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  1. Kinanah Yaseen
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Correspondence to Kinanah Yaseen.

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Yaseen, K., Herlitz, L.C. & Villa-Forte, A. IgA Vasculitis in Adults: a Rare yet Challenging Disease. Curr Rheumatol Rep 23, 50 (2021). https://doi.org/10.1007/s11926-021-01013-x

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