Aortitis is a general and nonspecific term that refers to a broad group of infectious and noninfectious conditions in which there is abnormal inflammation of the aortic wall. These inflammatory conditions have different clinical and morphologic features and variable prognoses. Some conditions exclusively affect the aorta, whereas others may also extend to its major branches. The clinical manifestations are often vague and nonspecific, including nonspecific pain, fever, malaise, elevated levels of acute phase reactants (e.g., serum C-reactive protein and erythrocyte sedimentation rate), and other systemic manifestations. As a result, aortitis is often overlooked during the initial workup of patients with constitutional symptoms and systemic disorders. In addition, organ ischemia and acute life-threatening aortic syndromes, such as aortic dissection or rupture, can complicate the course in some patients.

A multimodality imaging approach is often required for assessment of both the aortic wall and aortic lumen. Imaging techniques include ultrasonography, echocardiography, multidetector computed tomography (CT), magnetic resonance imaging (MRI), nuclear medicine imaging with fluorine 18-fluorodeoxyglucose (18F-FDG) positron emission tomography/CT fusion (PET/CT) or gallium 67 (67GA), and conventional angiography. Each of these modalities has strengths and limitations. Regardless of the imaging modality used, changes in the aortic wall and aortic lumen must be evaluated. Imaging is also required for surveillance of disease activity and treatment planning.

Classification

There are various classifications, but a simple classification of aortic inflammation into two broad categories (noninfectious and infectious) is practical and clinically useful ( Box 138.1 ). Noninfectious aortitis can be subdivided into large-, medium-, and small vessel vasculitis because diseases in this category commonly affect other vessels and may be part of a systemic disorder. It is essential to differentiate between noninfectious causes of aortitis and the much rarer infectious causes because the treatment differs greatly.

BOX 138.1
Classification of Aortitis

Noninfectious Aortitis

Large-Vessel Vasculitides

  • Giant cell arteritis (especially in older patients)

  • Takayasu arteritis (especially in younger patients)

  • Immunoglobulin G4–related aortitis

  • Rheumatoid arthritis

  • Systemic lupus erythematosus

  • Ankylosing spondylitis

  • Reiter syndrome

  • Cogan syndrome

Medium- and Small-Vessel Vasculitides

  • Wegener’s arteritis

  • Polyarteritis nodosa

  • Behcet disease

  • Relapsing polychondritis

  • Sarcoid

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