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Published in: Current Rheumatology Reports 10/2022

Open Access 03-08-2022 | Primary Immunodeficiency | Vasculitis (C Dejaco and C Duftner, Section Editors)

Spectrum of Large- and Medium-Vessel Vasculitis in Adults: Neoplastic, Infectious, Drug-Induced, Autoinflammatory, and Primary Immunodeficiency Diseases

Authors: Fabian Lötscher, Roxana Pop, Pascal Seitz, Mike Recher, Luca Seitz

Published in: Current Rheumatology Reports | Issue 10/2022

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Abstract

Purpose of Review

To provide a comprehensive review of drugs and neoplastic, infectious, autoinflammatory, and immunodeficiency diseases causing medium- to large-vessel vasculitis in adults with emphasis on information essential for the initial diagnostic process.

Recent Findings

Entities with medium- to large-vessel vasculitis as clinical manifestations have been described recently (e.g., adenosine deaminase-2 deficiency, VEXAS-Syndrome), and vasculitis in established autoinflammatory or immunodeficiency diseases is increasingly being identified.

Summary

In the diagnostic process of medium- to large-vessel vasculitis in adults, a large variety of rare diseases should be included in the differential diagnosis, especially if diagnosis is made without histologic confirmation and in younger patients. Although these disorders should be considered, they will undoubtedly remain rare in daily practice.
Appendix
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Metadata
Title
Spectrum of Large- and Medium-Vessel Vasculitis in Adults: Neoplastic, Infectious, Drug-Induced, Autoinflammatory, and Primary Immunodeficiency Diseases
Authors
Fabian Lötscher
Roxana Pop
Pascal Seitz
Mike Recher
Luca Seitz
Publication date
03-08-2022
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 10/2022
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-022-01083-5
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