Published in:
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
Login to get access
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.