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Updated: Jun 1, 2026

An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
The pathology of large-vessel vasculitides
Dylan V Miller1, Joseph J Maleszewski
1Intermountain Medical Center & University of Utah, Murray, USA. dylan.miller@imail.org
Insights
This review details the pathology of large-vessel vasculitis affecting the aorta and its branches. It covers conditions like giant cell arteritis, Takayasu arteritis, and inflammatory aneurysms, focusing on their morphological features.
Area of Science:
- Pathology
- Vascular Medicine
- Rheumatology
Background:
- Large-vessel vasculitis affects the aorta and its main branches.
- It encompasses diseases such as giant cell arteritis and Takayasu arteritis.
- Rarely, it can be a complication of other autoimmune and infectious diseases.
Purpose of the Study:
- To review the morphologic pathology of large-vessel vasculitis.
- To focus on specific conditions including giant cell arteritis, Takayasu arteritis, and idiopathic aortitis.
- To discuss lymphoplasmacytic aortitis and inflammatory aneurysms, including their association with IgG4-related conditions.
Main Methods:
- This is a review article, synthesizing existing literature.
- Focuses on morphologic and pathological aspects.
- Integrates recent findings on IgG4-related aortitis and inflammatory aneurysms.
Main Results:
- Large-vessel vasculitis presents diverse pathologies.
- Giant cell arteritis and Takayasu arteritis are primary forms.
- IgG4-related aortitis and inflammatory aneurysms represent a distinct, emerging category.
Conclusions:
- Understanding the morphology of large-vessel vasculitis is crucial for diagnosis and management.
- The spectrum of these diseases is broad, including autoimmune, infectious, and IgG4-related conditions.
- Further research into IgG4-related aortitis and inflammatory aneurysms is warranted.
Abstract:
Vasculitis affecting large elastic arteries, including the aorta and major proximal branches, encompasses various diseases including Takayasu arteritis, giant cell (or temporal) arteritis, and tertiary syphilis, but also may occur as a rare complication of Behçet's disease, rheumatoid arthritis, sarcoidosis, Cogan syndrome, Kawasaki disease, ankylosing spondylitis, systemic lupus erythematosus and Wegener's granulomatosis. Recent reports have also established a link between inflammatory abdominal aortic aneurysm as well as lymphoplasmacytic thoracic aortitis with an overabundance of IgG4-producing plasma cells and the burgeoning constellation of 'Hyper-IgG4' syndromes. This review focuses on morphologic aspects of large-vessel vasculitis pathology associated with giant cell arteritis, Takayasu arteritis, idiopathic or isolated aortitis, lymphoplasmacytic thoracic and ascending aortitis, and the inflammatory aneurysm/retroperitoneal fibrosis syndrome.
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