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European Journal of Echocardiography Advance Access published online on May 13, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen157
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Horton's aortitis

Vanessa Marchal* and Muriel Sprynger

CHU de Liège, Sart Tilman B35, Liège 4000, Belgium

Received 8 November 2007; received in revised form 12 April 2008; .

* Corresponding author. Tel: +32 498753676; fax: +32 43714416. E-mail address: vanessa{at}produweb.be


   Abstract
Top
 Abstract
Introduction
 Case report
 Discussion and conclusion
 References
 
Giant cell arteritis is the most common systemic vasculitis in people over the age of 50 years. Ischaemic manifestations are well known. ‘Occult’ manifestations as aortic aneurysmal disease need consideration. The incidence of aortic aneurysm and/or dissection is about 18.5 per 1000 person-years at risk (18.9 in Lugo(4) and 18.7 in Olmsted County(3)). Predictive factors are hypertension, polymyalgia rheumatica, coronaropathy, and hyperlipaemia. Another factor is the apparition of an aortic regurgitation murmur as in this case. So, these patients should be monitored by echocardiography.

Keywords: Horton; Aneurysm; Multinucleated giant cell


   Introduction
Top
 Abstract
 Introduction
Case report
 Discussion and conclusion
 References
 
Giant cell arteritis (GCA) is a systemic vasculitis that affects large- and medium-sized arteries in patients of more than 50 years.1,2 Annual prevalence is 15–33 cases per 100.000 person aged 50 years and older. The aorta and any of its branches can be affected. The aortic wall is primarily concerned. In case of aneurysm, the dilatation of the sinuses of Valsalva can cause valvular separation and thus induce regurgitation.

Giant cell arteritis' aortitis is sometimes neglected and can lead to acute aortic dissection and/or aortic aneurysm in about 18% of the cases.3,4

We report the case of a thoracic aneurysm that was incidentally discovered after 13 years of corticotherapy for GCA while investigating an aortic regurgitation murmur.


   Case report
Top
 Abstract
 Introduction
 Case report
Discussion and conclusion
 References
 
In December 2005, a 75 year old women took cardiologic advice for shortness of breath. Her medical history included chronic obstructive pulmonary disease, giant cell arteritis proved by a temporal artery biopsy, polymyalgia rheumatica (PMR) (diagnosed in 1992), recent hypertension (diagnosed in 2006), and microscopic hematury. Cardiovascular risk factors are hypertension and smoking (20 cigarettes per day). Her daily treatment consisted of Tibolone, N-acetylcystine, and methylprednisolone 2 mg every 2 days. Physical examination revealed a normal blood pressure (140/70 mm Hg), a regular heart rate (65 per min) and a mild parasternal diastolic murmur (grade 2/6). Some rhonchi were heard with disseminated wheezing. The abdominal examination was normal. The patient's weight was 55 kg and her height 150 cm. She had a good physical condition and no signs suggesting relapsing of GCA. The electrocardiogram was normal. Biology showed a C-reactive protein value of 2.3 mg/L, Hb of 12.7 g/dL, leucocytes 6480 per mm3, erythrocytes sedimentation rate (ESR) 9 mm/1 h, fibrin 4 g/L, and no antineutrophils antibodies (ANCA). The transthoracic echocardiography showed a normal left ventricle systolic function, mild aortic regurgitation with pressure half-time of 400 ms, a moderate mitral regurgitation, and a tricuspid regurgitation (grade 2–3/4), with a mild pulmonary hypertension (systolic pulmonary pressure: 34 mm Hg). Abdominal aorta and renal arteries were normal. Ascending aorta was dilated just above the Valsalva's sine (maximal diameter: 55.5 mm). The pericard was virtual (Figure 1).


Figure 1
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  		Figure 1 Echocardiography: TM mode: ascending aorta aneurysm.

 
The thoracic aorta diameter was confirmed by CT-Scan (Figure 2). The preoperative assessment was completed by a coronarography which revealed a monotroncular atherosclerotic coronary disease and confirmed the aortic dilatation. The ascending aorta was preventively replaced by a 28 mm ‘Vascutex’ conduit, completed by a venous bypass on the posterior interventricular artery. The aortic valve was preserved after plicature of the sine of Valsalva and suspension of the corners. The corticotherapy was continued. The shortness of breath regressed with the optimization of chronic obstructive pulmonary disease treatment. The patient's evolution was uneventful.


Figure 2
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  		Figure 2 CT scan: ascending aorta aneurysm.

 
Histological examination of the aortic wall samples (6.5 x 5 x 0.4 cm) assessed the diagnosis of GCA. The aortic internal wall was macroscopically congestive with mild lipid depositions and the external wall was irregular. Histological examination showed fibrosis and thickening of the intima and the media. The media contained an inflammatory infiltrate with lymphocytes, plasma cells, and multinucleated giant cells. Within the fibrosed adventitia, these inflammatory cells formed regular lymphoid nodes (Figure 3).


Figure 3
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  		Figure 3 Multinucleated giant cells and fibrosed media.

 

   Discussion and conclusion
Top
 Abstract
 Introduction
 Case report
 Discussion and conclusion
References
 
This clinical case enhances the need of looking for aortic aneurysm in the follow-up of GCA. This adverse evolution is not as rare as one could think. It was confirmed by two studies carried out by Nuenninghoff et al. and Gonzales-Gay et al. Annual prevalence is 15–33 cases per 100.000 person aged 50 years and older. The histological examination showed fibrosis and thickening of the intima, the media,5 and the adventitia6 with loss of normal elastic fibres and a chronic inflammatory cell infiltrate involving the full thickness of the wall5 with many multinucleated giant cells, lymphocytic, and plasma cellular infiltrates. In our patient, giant cell aortitis was histologically confirmed. There are many clinical manifestations of giant cell's aortitis: aortic aneurysm, aortic dissection, aortic rupture, aortic arch syndrome, aortic wall haematoma, aortic regurgitation, aortopulmonary, or aortodigestive fistula.7 However, some clinical signs are well correlated with the higher incidence of aneurysms3,4: systemic hypertension, PMR, coronaropathy, and hyperlipaemia. The most predictive association is, nevertheless, essential hypertension and PMR with an acute severe inflammatory response at the time of diagnosis. Another predictive factor is the apparition of an aortic regurgitation murmur as in this case. The aortic regurgitation is due to the dilatation of the sinuses of Valsalva which causes the aortic valve commissure separation.7,8 Aortitis is often diagnosed in patients with well-known GCA, but most of the time when the corticoïds are progressively lowered or even when they are stopped.7–13 Therefore, the thoracic aorta aneurysm is often lately diagnosed. The mean delay between GCA's diagnosis and discovery of the thoracic aneurysm varies from 5 to 7 years.1,7 The normalization of the sedimentation rate does not exclude the evaluative capacities of the aortitis.8–10,13,14 The ongoing inflammatory process could explain the late expression of the disease.7

The originality of this case is the extremely late expression of the aortic disease (diagnosis of aortitis 13 years after the diagnosis of GCA, despite of a non-regressive corticoïd treatment). Many aetiologies could be evoked: infection anevrysm (syphilis, salmonella, etc.), inflammatory non-septic aneurysm, Marfan's syndrome, Takayasu's disease, Cogan's syndrome, Behcet's syndrome, and spondylarthropathies.15 In our case, the confirmation was histological. Giant cell aortitis can lead to aneurysmal evolution, to dissection, aortic rupture, and sudden death. Those issues are due to elastophagia and elastolysis of the arterial wall,16,17 acute aortitis of the whole wall, and vasa vasorum arteritis. Patients with giant cell arteritis require a long-term follow-up because of the risk of aortic dissection and sudden death. Lots of authors recommend a yearly lateral chest radiography.8,10,14,18–20,22

However, the use of transthoracic echocardiography (eventually completed by CT-scan, MRI, and PET scan) is much effective. Potential advantages are no irradiation, good sensitivity and specificity, low cost, and easy availability.1,15,21 Particular attention must be paid to patients with arterial high blood pressure, hyperlipaemia, and/or new aortic regurgitation murmurs. Prospective imaging studies are needed1 in order to elaborate the guidelines of the follow-up of GCA.


   References
Top
 Abstract
 Introduction
 Case report
 Discussion and conclusion
 References
 

  1. Agard C, Ponge T, Fradet G, Baron O, Sagan C, Masseau A, et al. Giant cell arteritis presenting with aortic dissection: two cases and review of the literature. Scand J Rheumatol (2006) 35:233–6.[CrossRef][Web of Science][Medline]
  2. Salvarini C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant cell arteritis. New Engl J Med (2002) 347:261–71.[Free Full Text]
  3. Nuenninghoff DM, Hunder GG, Christianson TJH, Mc Clelland RL, Matteson EL. Incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis. A population-based study over 50 years. Arthritis Rheum (2003) 48:3522–31.[CrossRef][Web of Science][Medline]
  4. Gonzalez-Gay MA, Garcia-Porrua C, Pineiro A, Pego-Reigosa R, Llorca J, Hunder GG. Aortic aneurysm and dissection in patients with biopsy-proven giant cell arteritis from northwestern Spain. A population-based study. Medicine (2004) 83:335–41.[CrossRef][Medline]
  5. Javed MA, Sheppard MN, Pepper J. Aortic root dilation secondary to giant cell aortitis in a human immunodeficiency virus-positive patient. Eur J Cardiothorac Surg (2006) 30:400–1.[Abstract/Free Full Text]
  6. Connery CP, Descalzi ME, Krishner R, Inflammatory aneurysm of the ascending aorta, an unreported entity. J Cardiovasc Surg (1994) 35:33–4.[Medline]
  7. Le Tourneau T, Millaire A, Asseman Ph, De Groote P, Théry C, Ducloux G. Aortite de Horton. Revue de littérature. Ann Med Intern (1996) 147:361–8.
  8. Evans JM, Bowles CA, Bjornsson J, Mullany CJ, Hunder GG. Thoracic aortic aneurysm and rupture in giant cell arteritis. A descriptive study of 41 cases. Arthritis Rheum (1994) 37:1539–47.[Web of Science][Medline]
  9. Penin F, Schmidt JM, Froment J, Cuny G. Dissection aortique et maladie de Horton. Deux cas. Ann Med Intern (1984) 135:153–7.
  10. Marchionni N, Di Bari M, Ferruci L, Moschi G, Dabizzi RP, Di Donato M, et al. Giant cell arteritis causing a severe aortic regurgitation secondary to aneurysm of the ascending aorta. A case report. Angiology (1987) 38:712–6.[Abstract/Free Full Text]
  11. Harris M. Dissecting aneurysm of the aorta due to giant cell arteritis. Br Heart J (1968) 30:840–4.[Free Full Text]
  12. Flynn JT. A 77 year old woman with giant cell arteritis, back pain, and shock. NY State J Med (1985) 85:502–8. (The CPC).[Web of Science][Medline]
  13. Salisbury RS, Hazleman BL. Successful treatment of dissecting aortic aneurysm due to giant cell arteritis. Ann Rheum Dis (1981) 40:507–8.[Abstract/Free Full Text]
  14. How J, Strachan RW. Aortic regurgitation as a manifestation of giant cell arteritis. Br Heart J (1978) 40:1052–4.[Abstract/Free Full Text]
  15. Blockmans D, Stroobants S, Maes A, Mortelmans L. Positron emission tomography in giant cell arteritis and polymyalgia rheumatica: evidence for inflammation of the aortic arch. Ann J Med (2000) 108:246–9.
  16. Costello JM, Nicholson WJ. Severe aortic regurgitation as a late complication of temporal arteritis. Chest (1990) 98:875–7.[CrossRef][Web of Science][Medline]
  17. Case reports of the Massachussets General Hospital. Case 21-1971. N Engl J Med (1971) 284:1201–10.[Web of Science][Medline]
  18. Klinkhoff AV, Reid GD, Moscovich M. Aortic regurgitation in giant cell arteritis. Arthritis Rheum (1985) 28:582–5.[CrossRef][Web of Science][Medline]
  19. Save-Söderberg J, Malmvall BE, Andersson R, Bengtson BA. Giant cell arteritis as a cause of death. Report of nine cases. JAMA (1986) 255:493–6.[Abstract/Free Full Text]
  20. Le Tourneau T, Asseman P, Klug D, Prat A, Bauchart JJ, Aisenfarb JC, et al. Fissuration d'un anévrysme thoracique au cours d'une maladie de Horton, évolution favorable après chirurgie. Arch Mal Coeur (1994) 87:1489–92.[Medline]
  21. Agard C, Ponge T, Hamidou M, Barrier JH. Role for vascular investigations in giant cell arteritis. Joint Bone Spine (2002) 69:367–72.[CrossRef][Web of Science][Medline]
  22. Cormier JM, Cormier F, Laridon D, Vuong PN. Maladie de Horton et anévrisme aortique: Coïncidence ou association? J Mal Vas (2000) 25:92–7.

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This Article
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