Aortitis and aortic aneurysm in systemic vasculitis

Published on Jul 27, 2011
· DOI :10.5772/18875
Ana García-Martínez23
Estimated H-index: 23
Sergio Prieto-González22
Estimated H-index: 22
+ 3 AuthorsMaria C. Cid51
Estimated H-index: 51
Vasculitis encompasses a heterogeneous group of disorders characterized by inflammation of blood vessels. Inflammation affects vessels of any type and size, and causes a wide range of clinical manifestations, depending on the vascular bed involved. The preferential size of involved vessels and the targeted tissues determine the clinical presentation and serve as key elements for classification (Watts & Scott, 2009). Vasculitis may occur as a primary process or may be secondary to an underlying disease such as infection, malignancy or other systemic autoimmune or chronic inflammatory diseases. Blood vessel inflammation results in abnormal vascular remodelling with the potential of severe clinical consequences. In some instances, inflammation leads to intimal hyperplasia resulting in vascular stenoses and ischemia of the tributary tissues. In other settings, inflammation causes disruption of the vessel wall architecture leading to aneurysm formation and eventual dissection or rupture. Aortitis is the term used to define inflammation of one or more layers of the aortic wall and may have an infectious or non-infectious origin (Gornik & Creager, 2008). Non-infectious aortitis is usually part of the spectrum of vascular involvement occurring in primary largevessel vasculitis including giant-cell arteritis (GCA) of the elderly and Takayasu’s arteritis (TAK). Aortitis is a major component of these diseases and may lead to severe complications including aortic aneurysm, dissection or stenosis. Aortitis may also present as a circumscribed condition named isolated aortitis. This term refers to aortitis incidentally found at the time of histopathological examination of aortas obtained from necropsy studies or from patients who have undergone surgical repair of aortic aneurysm or aortic valve replacement. Existing studies are retrospective and most patients have not been prospectively and systematically evaluated in search for a systemic vasculitis or other chronic inflammatory diseases. There is some controversy about whether isolated aortitis is a specific condition or represents an incomplete view of a systemic disease. Occasionally, aortitis may occur in the setting of other primary systemic vasculitis, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (Chirinos et al., 2004; Lee et al., 2008), and other autoimmune disorders or chronic inflammatory diseases (i.e. sarcoidosis, Crohn’s disease, ankylosing spondylitis, Behcet’s disease, Cogan’s disease, and IgG4-related disease) (Domenech et al., 2005; Gluth et al., 2006; Palazzi et al.,
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  • Citations (3)
#1Carlo PalazziH-Index: 19
#2Carlo SalvaraniH-Index: 73
Last. Ignazio OlivieriH-Index: 54
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Abstract Aortic involvement is a potential life-threatening complication of ankylosing spondylitis, usually occurring late in the course of this frequent disease. Inflammatory lesions evolving to fibrosis are primarily localized in the aortic root causing regurgitation, but this process can extend into the left atrium (subaortic bump) involving the mitral valve and the heart conduction system. First, second and third degree atrioventricular blocks are the most common conduction alterations descr...
32 CitationsSource
#1Ralf KolvenbachH-Index: 19
#2Ron KarmeliH-Index: 3
Last. Markus Meyer-GaessnerH-Index: 1
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Background Endovascular treatment of the ascending aorta is particularly challenging because of the anatomic features of this aortic segment. Only patients without connective tissue disorders, clinically relevant aortic regurgitation or stenosis, or concomitant coronary artery disease can be considered for an endovascular procedure. We report our results in a series of patients with aneurysms or intramural hematoma, penetrating ulcers, or floating thrombus who were scheduled for stent grafting. ...
56 CitationsSource
Purpose of reviewIn last few years, there have been significant advances in our understanding of a newly recognized condition known as IgG4-related systemic disease. This review will focus on IgG4-related systemic disease as a cause of thoracic aortitis, inflammatory abdominal aortic aneurysm or per
160 CitationsSource
#1Kimberly P. Liang (University of Pittsburgh)H-Index: 10
#2Vaidehi ChowdharyH-Index: 13
Last. Kenneth J. Warrington (University of Pittsburgh)H-Index: 36
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Objective. To identify the clinical presentation and histopathologic characteristics of noninfectious ascending aortitis. Methods. A retrospective medical record and histopathology review was performed of patients with histologic evidence of active noninfectious aortitis who underwent ascending aortic aneurysm resection at Mayo Clinic between January 1, 2000, and February 28, 2006. Clinicopathologic features were recorded, including demographics, clinical presentation, laboratory, imaging findin...
50 CitationsSource
#1Richard A. Watts (UEA: University of East Anglia)H-Index: 50
#2David G. I. Scott (NNUH: Norfolk and Norwich University Hospital)H-Index: 34
The systemic vasculitides are a group of multisystem diseases characterized by inflammation of blood vessels. The aetiopathogenesis is unknown, and therefore nomenclature and classification are often descriptive and based on pathological features. Generally agreed classification schemes are vital to enable large multicentre or multinational clinical trials to be undertaken. An algorithm has recently been developed to harmonize use of the American College of Rheumatology (ACR) 1990 criteria and t...
42 CitationsSource
#1Maria C. CidH-Index: 51
Last. Ana García-MartínezH-Index: 23
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Although repeatedly reported in the literature, the extracranial involvement by giant-cell arteritis has been considered anecdotal until recent years. The emergence of new or improved imaging techniques along with a closer follow-up of these patients and their increase in life expectancy are beginning to underline that the clinical impact of extracranial involvement by GCA may be more relevant than previously thought. This review focuses on the extent of vascular involvement in GCA as reported b...
35 CitationsSource
#1Gabriella Nesi (UniFI: University of Florence)H-Index: 37
#2Chiara Anichini (UniFI: University of Florence)H-Index: 5
Last. Franca Gori (UniFI: University of Florence)H-Index: 9
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A total of 338 surgical specimens of the thoracic aorta were studied morphologically. These were obtained from patients (238 males, 100 females) operated on for aortic aneurysm or dissection at the Careggi Hospital, Florence, Italy, between January 1999 and June 2005. Medial degeneration was diagnosed in 299 cases (138 aneurysms, 161 dissections), atherosclerosis with extensive medial destruction was seen in 32 cases (26 aneurysms, 6 dissections), and giant cell arteritis (GCA) was found in 7 ca...
29 CitationsSource
86 CitationsSource
#1Chetan MukhtyarH-Index: 17
#2Loïc Guillevin (University of Paris)H-Index: 98
Last. Raashid Luqmani (University of Oxford)H-Index: 54
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Objectives: To develop European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis. Methods: An expert group (10 rheumatologists, 3 nephrologists, 2 immunolgists, 2 internists representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search through a modified Delphi technique. In accordance with standardised EULAR operating procedures, r...
395 CitationsSource
Purpose of reviewUltrasonography, MRI, and PET are increasingly studied in large-vessel vasculitis. They have broadened our knowledge on these disorders and have a place in the diagnostic approach of these patients.Recent findingsTemporal artery ultrasonography can be used to guide the surgeon to th
131 CitationsSource
Cited By3
#1Anjali Prasad (Mayo Clinic)H-Index: 1
#2Meghana R.K. Helder (Mayo Clinic)H-Index: 7
Last. Hartzell V. Schaff (Mayo Clinic)H-Index: 107
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Background Patients with active aortitis who undergo repair of ascending aortic aneurysms have an increased risk of late reoperation and decreased late survival. We aimed to determine the reasons for these poor outcomes and the influence of medical management. Methods We reviewed records of 186 patients (median age 73.9 years; 120 women) with noninfectious aortitis after elective ascending aortic aneurysm repair (January 1955 through December 2012). Landmark analysis was used to compare outcomes...
#1Cătălina Arsenescu-Georgescu (Grigore T. Popa University of Medicine and Pharmacy)H-Index: 1
#2Liviu Macovei (Grigore T. Popa University of Medicine and Pharmacy)H-Index: 1
Abstract Vasculitis is inflammation of blood vessel walls. Vasculitis may occur as a primary process or may be secondary to an underlying disease such as infection, malignancy, or other systemic autoimmune or chronic inflammatory diseases. These diseases are often associated with organ- and life-threatening manifestations. The vasculitis is generally grouped by the size of the predominant vessel involved with the main categories including small-, medium-, and large-vessel vasculitis and vasculit...
#1Ana García-Martínez (University of Barcelona)H-Index: 23
#2Pedro Arguis (University of Barcelona)H-Index: 14
Last. Maria C. Cid (University of Barcelona)H-Index: 51
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Background Aortic structural damage (ASD) may complicate the course of patients with giant cell arteritis (GCA). However the frequency and outcome of ASD has not been assessed in long term prospective studies. Methods In a previous screening of 54 biopsy proven GCA patients, significant ASD was detected in 12 (22.2%) after a median follow-up of 5.4 years. These patients were periodically evaluated (every 4 years) over a median of 10.3 years (range 4–16.6 years) in order to investigate the develo...
56 CitationsSource