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Inflammatory Diseases of the Coronary Arteries

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PanVascular Medicine

Abstract

Rarely, inflammatory disorders affect the coronary arteries, as well as the aorta and other large arteries. Giant cell arteritis (GCA), Takayasu’s arteritis (TA), polyarteritis nodosa (PAN), and Kawasaki’s disease (KD) are the major forms of systemic vasculitis that most often target these vessels. However, vasculitis is a much less common cause of coronary artery disease, aortic aneurysm, and large-artery stenosis than atherosclerosis, and the two are often difficult to distinguish from each other based on clinical and imaging findings alone. Vasculitic disorders are usually associated with signs and symptoms of multiorgan system involvement as well as laboratory findings of systemic inflammation, but it is not always the case. For example, a patient with GCA will often present with headache, scalp tenderness, transient visual obscuration, and fatigue and elevated serum levels of acute-phase reactants, while a minority may only present with aortic aneurysm and limb claudication without the typical clinical features. Patients with TA who develop pulseless disease will often have systemic complaints, but others may display only the signs and symptoms related to the involved arteries. Kawasaki’s disease evokes the appropriate suspicion of coronary artery vasculitis in children who present with the typical features of fever, mucocutaneous lesions, and cervical LN enlargement. Atypical clinical presentations, although uncommon, may be more of a diagnostic challenge. While the clinical presentation, laboratory findings, and the results of imaging studies may raise the possibility of systemic vasculitis, the gold standard for diagnosis is tissue histopathology. Therefore, a tissue diagnosis should be pursued if possible. However, tissue biopsy may not be feasible in some cases and the diagnosis reached from the clinical, laboratory, and imaging data alone. Depending on the nature of the disease, treatment usually consists of corticosteroids and other immunosuppressive agents and may include surgical intervention to manage the vascular complications.

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Abbreviations

Aneurysm:

A localized, blood-filled bulge in the wall of a blood vessel.

Antineutrophil Cytoplasmic Antibody:

An autoantibody directed against antigens in the cytoplasm of neutrophil; often seen in the small vessel vasculitides.

Antiphospholipid Antibody:

An autoantibody directed against anionic phospholipids in plasma membranes; associated with a clotting syndrome of the same name.

C-Reactive Protein:

An acute-phase protein produced by the liver, elevation of which is a marker of systemic inflammation.

Dendritic Cell:

A professional antigen presenting cell of the innate immune system with branched projections (dendrites).

Erythrocyte Sedimentation Rate:

A laboratory test using anticoagulated blood which measures the rate at which red blood cells fall and is measured and reported in mm/h. It is an indirect measure of pro-sedimentation factors such as fibrinogen and immunoglobulins. Increase in the rate at which red cells fall reflects elevation in these factors and is an indirect measure of systemic inflammation.

Interferon Gamma:

A dimerized proinflammatory cytokine which is produced by natural killer cells and Th1 cells.

Interstitial Keratitis:

Corneal scarring due to chronic inflammation of the corneal stoma; seen in a variety of infectious and inflammatory conditions such as syphilis and Cogan syndrome.

Leukocytoclastic Vasculitis:

Cutaneous inflammation of small blood vessels.

Libman-Sacks Endocarditis:

Nonbacterial endocarditis seen in systemic lupus erythematosus.

Major Histocompatibility Complex:

A set of cell surface molecules which bind to and facilitate presentation of antigen to the adaptive immune system.

Mononeuritis Multiplex:

Simultaneous inflammation of noncontiguous nerve trunks affecting at least two separate nerve areas.

Mucocutaneous Inflammation:

Erythema, pain, and sometimes ulceration of the mucosa and surrounds epidermis.

Multinucleated Giant Cells:

A mass formed by the union of distinct cells (usually macrophages).

Natural Killer Cells:

An innate cytotoxic immune cell, involved in killing infected or transformed cells which have downregulated MHC class 1. These cells also participate in the immune response by producing cytokines such as interferon gamma.

Pulse Steroids:

High doses of corticosteroids (0.5–1 g/day) given over several days.

SLE Disease Activity Index Instrument Score (SLEDAI):

A measure of lupus activity used in clinical trials.

T-Helper Cell:

A class of predominantly CD4 positive T cell which is involved directing the immune response.

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  1. Division of Rheumatology and Immunology, DUMC Duke Medicine, 2918, Durham, NC, 27710, USA

    John R. Dye, Mala S. Kaul & E. William St. Clair

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    Peter Lanzer

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Dye, J.R., Kaul, M.S., St. Clair, E.W. (2014). Inflammatory Diseases of the Coronary Arteries. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37393-0_64-1

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