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.
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| http://dx.doi.org/10.12659/ajcr.898673 | DOI Listing |
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A rare case of IgG4-related aortitis in the thoracic aorta mimicking an intramural hematoma: navigating the diagnostic labyrinth.
J Cardiothorac Surg
October 2024
Division of Cardiothoracic Surgery, The Valley Hospital, 223 N Van Dien Ave, Ridgewood, NJ, 07450, USA.
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- A 54-year-old woman was admitted with recurrent abdominal pain and new chest pain, leading to the discovery of a thoracic aortic aneurysm, suspected to involve intramural hematoma or aortitis.
- Initial conservative treatment was followed by surgical intervention after imaging suggested intramural hematoma, but surgery revealed severe aortitis without hematoma, indicating complex diagnostic challenges.
- Further examination identified IgG4-related disease linked to chronic lymphocytic leukemia (CLL), highlighting the need for a multidisciplinary approach and various diagnostic methods to accurately assess aortic conditions.
Coronary artery involvement in a patient with IgG4-related disease.
Radiol Case Rep
October 2023
Children`s Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
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- IgG4-RD is a rare chronic disorder characterized by high levels of IgG4-positive plasma cells affecting various organs, with rare coronary artery involvement.* -
- A 15-year-old female patient exhibited severe heart issues, including a significantly reduced ejection fraction and inflammation in cardiac walls, confirmed through cardiac MRI and coronary CT angiography.* -
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Necrotizing plasma cell-rich aortitis and sudden cardiac death: Late sequelae of COVID-19?
Cardiovasc Pathol
November 2023
Department of Pathology, University of Texas Medical Branch (UTMB), Galveston, Texas, USA.
The ongoing epidemic caused by the coronavirus SARS-CoV-2 is characterized by a variety of pathologic processes within the syndrome of COVID-19. Usually beginning as an upper respiratory infection with potential progression to a pneumonitis, many cases of COVID-19 that show minimal signs or symptoms initially may develop adverse systemic sequelae later, such as widespread thrombo-embolic phenomena, systemic inflammatory disorders (especially in children), or vasculitis. Here, we present a patient who suffered a sudden cardiac death following persistent SARS-CoV-2 viral positivity for four-and-one-half months after a mild clinical viral course.
View Article and Find Full Text PDFPachymeningitis associated with IgG4-related disease and ANCA positivity: Case report and review of the literature.
Autoimmun Rev
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Department of Internal Medicine and Clinical Immunology, Lariboisière Hospital, Université Paris Cité, 75010 Paris, France. Electronic address:
Objective: Hypertrophic pachymeningitis is a rare clinical disorder involving localized or diffuse thickening of the dura mater. Considering pachymeningitis is both in the clinical spectrum of IgG4-RD and ANCA vasculitis (specifically granulomatosis with polyangiitis), an overlap syndrome is discussed.
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A case of aortoduodenal fistula caused by IgG4-related periaortitis.
Mod Rheumatol Case Rep
January 2023
Department of Rheumatology, Kobe City Medical Center General Hospital, Kobe, Hyogo, Japan.
An 86-year-old man who underwent endovascular aortic repair for impending rupture of an abdominal aortic aneurysm a year ago presented to our hospital because of fatigue and black stools. Multiple bacterial specimens were detected in blood cultures, and computed tomography following oral administration of gastrografin demonstrated gastrografin in the abdominal aorta. The diagnosis of aortic duodenal fistula was confirmed and emergency abdominal aortic replacement was performed.
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