(I-3A.2) Giant cell aortitis of the ascending aorta without signs and symptoms of systemic vasculitis Prof. Podolec P. MD, PhD, Stępniewski J, MD, Drabik L. MD, Prof. Ereminienė E. MD, PhD, Prof. Benetis R. MD, PhD, Dziedzic H. MD

Keywords: ascending aorta, giant cell aortitis

Background
Giant cell arteritis (GCA) is a chronic inflammatory disease of the medium and large elastic arteries, especially cranial vessels, that predominantly occurs in the elderly. The estimated incidence in Europe in individuals over 50 years of age varies between 32 and 290/million/year [1] Aortic aneurysm or dissection develops only in up to 18% of patients with GCA. Diagnosis is often delayed due to poor recognition of early nonspecific symptoms and the management of these patients is under discussion [2].

Case description
This 71-year-old white Caucasian woman referred to the Cardiac Surgery Department because of the aneurysm of the ascending aorta complaining of weakness, headache and shortness of breath at exertion. She had more than 20-year history of arterial hypertension and was on medical treatment with ACE inhibitors and beta-adrenoblockers. Physical examination revealed normal symmetrical peripheral pulse, regular heart rate, equal extremity blood pressure 150/90 mmHg, no carotid and subclavian bruits, no clinical signs of heart failure. ECG: Sinus rhythm, LVH. Biochemical analysis: CRP – 3,94 mg/l, ESR – 18 mm/h. Test for syphilis – negative. Echocardiography: Aneurysm of ascending part (56 mm) and slightly dilated proximal part of aortic arch (39 mm), tricuspid aortic valve with II-III degree of aortic regurgitation (functional type I), LVEDD – 48 mm, LV hypertrophy, preserved LV systolic function. Computed tomography confirmed the echo results. Angiography: normal coronary arteries. The patient underwent valve sparing surgery – prosthesis of the ascending aorta and proximal part of arch (No. 26) and reconstruction of aortic valve. Pathophysiological examination of the aortic wall revealed inflammatory lesions that began in the adventitia with the signs of vascular injury, signs of medial necrosis with inflammatory cells, Langerhans and non Langerhans type giant cells and intimal proliferation.
The postoperative period was normal with subfebrile temperature about 1 week after surgery, stable hemodynamic parameters. The biochemical analysis showed slow reduction of CRP and ESR, normal values of anticoagulation. Medical treatment after surgery: Warfarin 5 mg/d (INR – 2,4) 3 months after surgery, aspirin 100 mg/d, immunosuppression with methylprednisolone 40 mg/d, metoprolol succ. 25-50 mg/d, perindopril/amlodipine 10/5 mg/d, spironolactone 25 mg/d, omeprazole 20 mg/d.

Guidelines
According to the management recommendations for large vessel vasculitis, developed by European League against Rheumatism (EULAR) in 2009, histopathological evidence is the gold standard for the diagnosis of giant cell arteritis, despite the normal ESR and C-reactive protein values. This diagnosis requires long term glucocorticoid therapy, the duration can extend to several years. These patients are at an increased risk of developing cardiovascular and cerebrovascular events, so the addition of low dose aspirin protects against such complications. There are no valid biomarkers for assessing response and diagnosing relapse in large vessel arteritis [1].

Even in the absence of signs and symptoms of systemic vasculitis, GCA of the ascending aorta is associated with an increased frequency of distal aortic events on long-term follow-up after surgery, so aortic imaging should be considered for monitoring long-term clinical outcomes in large vessel disease [1,3].

References
1. Mukhtyar C., Guilevin L. , M.C. Cid, B. Dasgupta, K. de Groot et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009; 68:318-323;
2. F.A. Borg, B. Dasgupta. Treatment and outcomes of large vessel arteritis. Best practice & Research Clinical Rheumatology 2009: 325-337;
3. He Wang, R. N. Smith, A.E. Spooner, E. M. Isselbacher, R. P. Cambria et al. Giant cell aortitis of the ascending aorta without signs or symptoms of systemic vasculitis is associated with elevated risk of distal aortic events. Arthritis & Rheumatism Accepted 2011

Expert’s conclusions:
It may be advised to gradually reduce the doses of steroids. According to rheumatology society guidelines 75 to 150 mg/d of ASA are recommended. Obviously, the inflammation markers such as CRP, ESR, WBC should be monitored regularly.
Giant cell aortitis of the ascending aorta without signs and symptoms of systemic vasculitis – flash presentation

Authors:
Prof. Podolec P. MD, PhD1, Stępniewski J, MD1, Drabik L. MD1, Prof. Ereminienė E. MD, PhD2, Prof. Benetis R. MD, PhD2, Dziedzic H. MD1

Experts:
Assoc. Prof. Kabłak-Ziembicka A.MD, PhD1, Prof. Andrzej Biederman MD, PhD3

1 Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John
Paul II Hospital, Krakow, Poland
2 Department of Cardiology, Department of Cardiac, Thoracic and Vascular Surgery, Medical
Academy, Lithuanian University of Health Sciences, Lithuania
3 Department of Cardiac Surgery, Institute of Cardiology, Warsaw, Poland
 

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