Inflammatory diseases of the aorta

Area of Science:

• Vascular Surgery
• Rheumatology
• Immunology

Background:

• Inflammatory aortic diseases encompass conditions like aortitis and periaortitis, potentially causing aneurysms, obstruction, rupture, and dissection.
• Large vessel vasculitis, including Takayasu arteritis and giant cell arteritis (GCA), affects all aortic wall layers, predominantly in the thoracic aorta.
• Behçet's disease can present with unusually shaped and located aortic aneurysms.

Purpose of the Study:

• To review the clinical presentation, diagnosis, and management of inflammatory aortic diseases.
• To highlight the association between periaortitis, inflammatory aortic aneurysms (IAA), and retroperitoneal fibrosis (RF).
• To discuss the role of IgG4-related disease in these conditions and treatment strategies.

Main Methods:

• Literature review of inflammatory aortic diseases, including aortitis, periaortitis, and related conditions.
• Analysis of diagnostic modalities, emphasizing vascular imaging and occasional biopsy.
• Evaluation of treatment approaches, including immunosuppression and surgical considerations.

Main Results:

• Diagnosis of aortitis often relies on vascular imaging, with biopsy sometimes necessary, especially for ascending aorta involvement.
• Periaortitis, often overlapping with RF, can lead to IAA and may be linked to IgG4-related disease in about 50% of cases.
• Immunosuppression, primarily with glucocorticoids, is standard for active inflammatory aortic diseases, but may be unnecessary post-surgery or in inactive disease.

Conclusions:

• Inflammatory aortic diseases require accurate diagnosis through imaging and sometimes biopsy.
• Management strategies vary, with immunosuppression being key for active disease, but potentially avoidable in specific scenarios.
• Conditions like periaortitis, IAA, and RF share overlapping pathology and may be associated with IgG4-related disease, influencing treatment decisions.