Etiology, Diagnosis and Management of Aortitis
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REVIEW
Etiology, Diagnosis and Management of Aortitis Sanjiv Sharma1 • Niraj Nirmal Pandey1 • Mumun Sinha1 • S. H. Chandrashekhara1
Received: 27 January 2020 / Accepted: 8 April 2020 Ó Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2020
Abstract Aortitis includes conditions with infectious or non-infectious etiology, characterized by inflammatory changes in one or more layers in aortic wall. Age at onset, geographic predilections, distribution and pattern of involvement in aorta, its branches and pulmonary arteries, and systemic associations provide a clue to etiology. Clinical presentations are often non-specific. An integrated approach including clinical, laboratory and imaging assessment is essential to confirm diagnosis and plan treatment. Assessment of disease activity is the key as it influences timing and outcome of treatment. Markers of activity include clinical, laboratory and imaging. Medical management remains the first-line therapy. Revascularization is indicated in the presence of hemodynamically significant stenosis and inactive disease. In the presence of flash pulmonary edema, left ventricular dysfunction or hypertensive encephalopathy, revascularization is performed irrespective of disease activity. Endovascular management is favored over surgery due to its high success and low restenosis rates. Symptomatic aneurysmal disease is usually managed by surgery. Keywords Aortitis Giant cell arteritis Takayasu arteritis Disease activity
& Sanjiv Sharma [email protected] 1
Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi 110029, India
Introduction Aortitis includes a broad group of clinical conditions that are characterized by inflammatory changes in one or more layers in the aortic wall. It may be of infectious origin, but is more frequently of non-infectious etiology, including immunological or connective tissue disorders [1]. Giant cell arteritis (GCA) and Takayasu arteritis (TA) are among the most common causes of aortitis, while less frequent etiologies include long-standing ankylosing spondylitis (AS), Cogan syndrome, relapsing polychondritis, Behcet’s disease, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) [2]. The clinical presentation is variable and non-specific, which makes the diagnosis of these conditions challenging. However, the age at onset, gender, geographic predilections, distribution and pattern of involvement within the aorta, involvement of its branches and pulmonary arteries, and systemic associations can provide a clue to the etiology. As an example, a ‘‘pulseless’’ pattern of involvement in a young woman of Asian origin is indicative of TA, whereas headache and jaw claudication in an elderly Caucasian woman would indicate GCA [3, 4]. Aortitis is often an unanticipated finding in patients being investigated for an unexplained fever, or chest, abdominal or back pain [1]. Given the nebulou
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