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Published on: February 4, 2021
Inflammatory ascending aortic disease: perspectives from pathology
1Division of Anatomic Pathology, Mayo Clinic, Rochester, Minn.
Abstract:
Inflammatory diseases of the aorta comprise a spectrum of disease with diverse clinical and histopathologic presentations. Broadly, they may be dichotomized into infectious and noninfectious varieties. Although uncommon, infectious forms, caused by bacteria, fungi, or mycobacteria, may result from hematogenous seeding of the aorta or direct spread from a contiguous infectious source. The noninfectious forms include a number of entities, the most common of which is atherosclerosis, a disease that primarily affects the aortic intima but has important secondary effects on the media and adventitia that may result in aneurysm formation. Other important noninfectious inflammatory diseases include giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis (Wegener granulomatosis), sarcoidosis, and lymphoplasmacytic aortitis. Importantly, there is increasing recognition that there is a subset of cases of lymphoplasmacytic aortitis perhaps better classified under the spectrum of so-called IgG4-related sclerosing disease, with important clinical and therapeutic ramifications. This review focuses on the variable and defining characteristics of the inflammatory aortopathies, specifically those affecting the ascending aorta, and discusses areas of important clinical and pathological distinction between them.
Insights
Inflammatory aortic diseases range from rare infections to common atherosclerosis. This review details various inflammatory aortopathies affecting the ascending aorta, aiding clinical and pathological distinctions.
Area of Science:
- Cardiovascular Pathology
- Immunology
- Vascular Medicine
Background:
- Inflammatory aortic diseases present diverse clinical and histopathologic features.
- Diseases are broadly categorized into infectious and noninfectious types.
- Infectious aortitis is uncommon, caused by bacteria, fungi, or mycobacteria.
Purpose of the Study:
- To review inflammatory diseases of the aorta, focusing on the ascending aorta.
- To highlight the defining characteristics of various inflammatory aortopathies.
- To discuss key clinical and pathological distinctions between these conditions.
Main Methods:
- Literature review of infectious and noninfectious inflammatory aortic diseases.
- Analysis of histopathologic and clinical presentations.
- Focus on ascending aorta involvement and differential diagnoses.
Main Results:
- Noninfectious forms include atherosclerosis, giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis, sarcoidosis, and lymphoplasmacytic aortitis.
- Atherosclerosis primarily affects the intima but impacts media and adventitia, potentially causing aneurysms.
- Lymphoplasmacytic aortitis may be associated with IgG4-related sclerosing disease, impacting treatment.
Conclusions:
- Understanding the spectrum of inflammatory aortopathies is crucial for accurate diagnosis.
- Distinguishing between infectious and noninfectious causes, and specific noninfectious types, is clinically significant.
- Recognition of IgG4-related sclerosing disease in aortitis has therapeutic implications.
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