Large vessel arteritis (Ackermann's Surgical Pathology, 8th Ed.)

There is a group of related nonsyphilitic diseases primarily affecting the aorta and its main branches and characterized by chronic inflammation and patchy destruction of the elements of the media. They may result in aortic insufficiency, diffuse aortic tortuosity and elongation, the aortic arch syndrome, aneurysm formation, and dissection of the vessel. They are more common in adults but have also been described in children.
In some aortic aneurysms, there is a thick outer wall composed of fibroblasts and collagen that entrap fat, nerves, and lymph nodes and that are accompanied by a heavy lymphoplasmatic infiltrate. These have been referred to as inflammatory aneurysms, but it is not clear whether they represent a distinct entity or simply a variant of atherosclerotic aneurysm, perhaps induced by antigens in the atheromatous plaque, and/or mediated by adhesion molecules; the latter possibility seems the most likely.
In the variety of arteritis known as Takayasu's disease,there is chronic inflammation and fibrosis of the arterial wall, which has a predilection for the aortic arch branches and results in the absence of pulses in the upper extremities, ocular changes, and neurologic symptoms. In later stages, superimposed changes of arteriosclerosis may obscure the diagnosis. In general, the possibility of an underlying arteritis should always be considered when arteriosclerotic changes in the aorta are seen in young or middle-aged individuals and when these changes are either segmental or occur at an unusual site. Most patients with Takayasu's disease are young, Asian, and female. The disease is rare in the United States, but it has been well documented. In a series of sixteen autopsy cases reported from South Africa, there was segmental coronary arteritis in three patients, with development of coronary aneurysms in two. There was co-existent tuberculosis in 37.5% of the patients, but this might well have been coincidental.
In Kawasaki's disease of infants, arterial changes are prominent and may result in sudden death from acute myocardial ischemia. Microscopically, the coronary and other arteries show reactive proliferative changes in the media, panarteritis, and frequent aneurysmal dilatation. Marked fibrosis is present in the healed stage. The etiology is unknown, but the pathologic changes are thought to be immune-mediated. Specifically, it has been suggested that the disease may be caused by a toxin that acts as a "superantigen."
Aortic arteritis can be seen associated with rheumatoid arthritis, ankylosing spondylitis, and scleroderma. There is a definite relationship between the type and location of the vessel and the frequency and etiology of the inflammatory diseases it may be affected by. Tuberculosis characteristically involves small vessels; rarely, it may be seen in larger vessels and results in stenosis or aneurysms.
Most of the reported cases of aneurysms of the superior mesenteric artery have been either syphilitic or mycotic; the majority of the latter were associated with bacterial endocarditis.

Selected Abstracts

1: Asian Cardiovasc Thorac Ann. 2007 Jun;15(3):252-4

High-risk repair of ascending aortic aneurysm due to giant cell aortitis.

Atluri P, Woo YJ.

, Division of Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania, Silverstein 4, 3400 Spruce Street, Philadelphia, PA 19104, USA. wooy@uphs.upenn.edu.

Giant cell arteritis increases the risk of developing a thoracic aortic aneurysm. Thoracic aortic aneurysm repair in octogenarians carries a profound increase in postoperative morbidity and mortality. We report the successful repair of an ascending aortic aneurysm in an 83-year-old woman with a history of treatment for temporal arteritis and pathologic evidence of giant cell aortitis.

 

2: Clin Exp Rheumatol. 2007 Jan-Feb;25(1 Suppl 44):S36-9

Giant-cell arteritis of the female genital tract associated with occult temporal arteritis and FDG-PET evidence of large-vessel vasculitis.

Bajocchi G, Zamorani G, Cavazza A, Pipitone N, Versari A, Boiardi L, Salvarani C.

Unità Operativa di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia, Italy. baiocchi.gianluigi@asmn.re.it

We describe a case of giant cell arteritis (GCA) of the female genital tract. Fluorine-18-fluorodeoxyglucose positron emission tomography (18F-FDG PET) and CT-scan showed evidence of large-vessel vasculitis involving the thoracic aorta and its branches, while temporal artery biopsy showed arteritis despite the absence of clinical manifestations suggestive of GCA. We review the literature and discuss the relationship between "cranial" GCA, large-vessel GCA and female genital GCA.


3: Clin Exp Rheumatol. 2007 Jan-Feb;25(1 Suppl 44):S15-7

The incidence of giant cell arteritis in Jerusalem over a 25-year period: annual and seasonal fluctuations.

Bas-Lando M, Breuer GS, Berkun Y, Mates M, Sonnenblick M, Nesher G.

The Hebrew University-Hadassah Medical School, Jerusalem, and Safra Hospital, Sheba Medical Center, Tel-Hashomer, Israel.

OBJECTIVE: Giant-cell arteritis (GCA) incidence is reported to be rising. A cyclic pattern of annual incidence rates and seasonal variations were reported by several groups. However, such fluctuations were not observed by others. We examined both annual and seasonal rates of GCA over a period of 25 years in Jerusalem. METHODS: Charts of all patients diagnosed as GCA between 1980-2004 were reviewed. In 170 cases GCA was biopsy-proven. Thirty-six additional cases were included as they met the American College of Rheumatology GCA classification criteria. Data on the Jerusalem population throughout the study period was collected from the annual publications of the Israel Bureau of Statistics. Age- and sex-specific incidence rates per 100000 population aged>or=50 were calculated. RESULTS: For the whole period, the average age-adjusted incidence rate was 11.3 per 100000, and 9.5 for the biopsy-positive cases. The female: male ratio was 1.4:1. Cyclic fluctuations of GCA incidence with 3 distinctive peaks, 8-10 years apart, were observed. Altogether, there was no apparent increase in GCA incidence during this period. Seasonal variations were observed: in 192 patients we were able to estimate the time of onset of GCA symptoms. It showed a peak in the months of May and June, with the number of patients being twice as expected for this period (p<0.001). CONCLUSION: GCA onset was more common in late spring and early summer, and fluctuations in GCA annual incidence with 3 distinctive peaks were observed during a 25-year period. These suggest infectious or other environmental etiology, however thus far no such agents were proven.

4: Presse Med. 2007 May;36(5 Pt 2):845-53. Epub 2007 Apr 3

Classification of systemic vasculatides

Guillevin L, Pagnoux C, Guilpain P.

Centre de référence national, Plan maladies rares, vascularites et sclérodermie systémique, Hôpital Cochin, AP-HP, Université Paris 5 - René Descartes, Paris, France. loic.guillevin@cch.aphp.fr

Vasculitides are defined by inflammation of blood vessel walls leading to vascular stenosis or occlusion, with various degrees of fibrinoid necrosis of the media and inflammatory infiltration, mainly neutrophilic and sometimes granulomatous. Various classifications of the vasculitides have been proposed. The classifications used most today are the 1990 American College of Rheumatology classification and the Chapel Hill nomenclature, published in 1994 . Only the latter distinguished between polyarteritis nodosa and microscopic polyangiitis and stressed the importance of antineutrophil cytoplasm autoantibodies (ANCA). In practice, primary systemic vasculitides are classified according to their clinical presentations, their precise histological features, and the size of the predominantly affected vessels. Some small-vessel vasculitides are associated with the presence of ANCA: 90% of patients with systemic Wegener's granulomatosis (mainly ANCA with cytoplasm labeling on indirect immunofluorescence and proteinase 3 specificity), 80% of the subjects with microscopic polyangiitis (mostly pANCA with myeloperoxidase specificity), and more than one third of those with Churg-Strauss syndrome (mostly pANCA).

5: Can J Ophthalmol. 2007 Feb;42(1):136-7.

Giant cell arteritis presenting with aortic aneurysm, normal erythrocyte sedimentation rate, and normal C-reactive protein.

Raja MK, Proulx AA, Allen LH.

Schulich School of Medicine, the Ivey Eye Institute, Department of Ophthalmology, University of Western Ontario, and St Joseph's Health Care, London, Canada.

CASE REPORT: We report on an extremely rare case of giant cell arteritis (GCA) presenting without elevated erythrocyte sedimentation rate or C-reactive protein level, with aortic root involvement, and in the absence of typical clinical findings. COMMENTS: The clinical signs and symptoms as well as the laboratory investigations involved in the diagnosis of GCA are discussed.


6: J Gen Intern Med. 2007 Jan;22(1):140-4.

Giant cell arteritis presenting as small bowel infarction.

Annamalai A, Francis ML, Ranatunga SK, Resch DS.

Department of General Internal Medicine, Southern Illinois University School of Medicine, Springfield, IL 62794-9636, USA. aannamalai@siumed.edu

Giant cell arteritis predominantly affects cranial arteries and rarely involves other sites. We report a patient who presented with small bowel obstruction because of infarction from mesenteric giant cell arteritis. She had an unusual cause of her obstruction and a rare manifestation of giant cell arteritis. In spite of aggressive therapy with steroids, she died a month later because of multiple complications. We discuss the diagnosis and management of small bowel obstruction and differential diagnosis of vasculitis of the gastrointestinal tract. We were able to find 11 cases of bowel involvement with giant cell arteritis in the English literature. This case report illustrates that giant cell arteritis can be a cause of small bowel obstruction and bowel infarction. In the proper clinical setting, vasculitides need to be considered early in the differential diagnosis when therapy may be most effective.


7: Fukuoka Igaku Zasshi. 2006 Dec;97(12):358-65.

Case of isolated thoracic aortic aneurysm as a manifestation of undiscovered giant cell arteritis.

Kishi T, Uchida T, Yasutsune T, Iwai T, Izumo A, Ohishi Y, Ohya M, Yamada A, Ando H, Okamatsu S.

Department of Cardiovascular Medicine, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.

A 73-year-old woman was referred to our hospital to investigate dilatation of an aortic arch which had been detected by a chest roentgenogram and severe aortic valve regurgitation detected by echocardiography. On admission, a computed tomography scan of the chest showed a large fusiform ascending aortic aneurysm. She had not shown any symptoms such as headache or polymyalgia rheumatica and had no significant coronary atherosclerosis. She underwent aneurysmectomy and reconstruction of the ascending aorta using cardiopulmonary bypass without aortic valve replacement, and pathological examination of the aneurismal wall revealed giant cell arteritis (GCA). Preoperatively, she did not have any temporal pain, and no signs of inflammation were detected serologically. Postoperatively, aortic valve regurgitation improved and she did well. However, three months after the surgery, she died suddenly due to the rupture or dissection of aorta. In the Japanese population, GCA is reportedly a rare cause of aortic aneurysm. However, retrospective studies show that GCA affects the aorta and that thoracic aortic aneurysm is a possible complication of GCA. In cases of the thoracic aortic aneurysms with unknown etiology, there is a possibility that GCA is the cause of the aortic aneurysm.


8: Front Neurol Neurosci. 2006;21:96-104.

Takayasu and temporal arteritis.

Schmidt WA.

Medical Center for Rheumatology Berlin-Buch, Berlin, Germany. w.schmidt@immanuel.de

Takayasu and temporal arteritis are primary large-vessel vasculitides. Ultrasound directly depicts the inflamed vessel wall, which is homogenously and circumferentially thickened. Furthermore, stenoses and occlusions occur. Ultrasound almost completely depicts the whole length of the common superficial temporal arteries, including the frontal and parietal ramus. Inflammation is often segmental. This may lead to a false-negative histology. The wall swelling is hypoechoic in acute temporal arteritis. It disappears within 2-3 weeks with corticosteroid treatment. Sonographers should use 8-15 MHz linear probes. The pulse repetition frequency should be about 2.5 kHz. Color box steering and beam steering should be maximal. It is essential that the color covers the artery lumen exactly. Sensitivities and specificities with regard to clinical diagnosis and histology are high. Large-vessel giant cell arteritis is a subset of temporal arteritis, with involvement of the subclavian, axillary, and proximal brachial arteries. The wall swelling resolves much slower with treatment. In Takayasu arteritis ultrasound is a valuable diagnostic tool to investigate particularly the common carotid, subclavian, and vertebral arteries. The echogenicity of the arterial wall thickening is, in general, higher than in giant cell arteritis, as the nature of Takayasu arteritis is more chronic, with less wall edema.


9: Rev Med Interne. 2007 Jun;28(6):363-70. Epub 2007 Jan 26.

Screening of abdominal aortic involvement using Doppler sonography in active giant cell (temporal) arteritis at the time of diagnosis. A prospective study of 30 patients

Agard C, Hamidou MA, Said L, Ponge T, Connault J, Chevalet P, Masseau A, Pistorius MA, Brisseau JM, Planchon B, Barrier JH.

Service de Médecine Interne, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44035 Nantes cedex 01, France. christian.agard@chu-nantes.fr

BACKGROUND: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis. PATIENTS AND METHODS: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta. RESULTS: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm. CONCLUSION: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

10: Br J Ophthalmol. 2007 Jun;91(6):715-8. Epub 2007 Jan 17