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Plasmacytic Aortitis with Occlusion of the Right Coronary Artery

Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Benjamin R. Zambetti, Edward Garrett, Jr.

Department of Cardiovascular Surgery, University of Tennessee Health Science Center, Memphis, TN, USA

Am J Case Rep 2016; 17:549-552

DOI: 10.12659/AJCR.898673

Available online:

Published: 2016-07-29

BACKGROUND: Inflammation of the aortic wall, known as aortitis, is a rare clinical entity which is frequently asymptomatic, or identified when the patient presents with an aortic aneurysm or dissection. It is most often caused by infection or autoimmune vasculitides such as giant cell or Takayasu’s arteritis.
CASE REPORT: The case presented is that of a 55-year-old man with symptomatic occlusion of the right coronary artery caused by a plasmacytic aortitis suggestive of IgG4 disease, which was successfully treated with coronary artery bypass grafting and an ascending aortic graft.
CONCLUSIONS: A review of the current literature emphasizes how poorly the etiology and natural history of plasmacytic aortitis is understood.

Keywords: Aortic Aneurysm, Thoracic - surgery, Aortitis - immunology, cardiac catheterization, computed tomography angiography, Coronary Occlusion - etiology, Coronary Vessels - diagnostic imaging, Diagnosis, Differential, Humans, Immunoglobulin G - immunology, Male, Middle Aged, Plasma Cells - immunology, Vascular Surgical Procedures - methods