Four cases of giant cell arteritis causing severe aortic regurgitation secondary to an aneurysm of the ascending aorta are described. In two cases, the nature of the aortic pathology could be suspected considering the past clinical evidence of temporal arteritis and/or polymyalgia rheumatica. In the two other cases, the cardiothoracic manifestations represented the onset of Horton disease.
Key words: aortic aneurysm; giant cell aortitis; Horton disease; vasculitis
Abbreviations: ESR = erythrocyte sedimentation rate; GCA = giant cell arteritis
Giant cell arteritis (GCA), or Horton disease, is a systemic vasculitis that primarily affects extracranial arteries of the head and neck in the elderly. It may present with signs and symptoms of a systemic disease and/or secondary to vascular ischemia. Headache, jaw claudication, fever, polymyalgia rheumatica, and an elevated erythrocyte sedimentation rate (ESR) are typical clinical features. The treatment of choice is therapy with corticosteroids, which usually prevent severe complications such as blindness.
With the lengthening of life expectancy in recent decades, GCA, as ascertained by a biopsy of the temporal artery, is on the increase. It is calculated that approximately 1% of the population > 50 years of age may be affected. (1,2) There also has been an increase in the number of reports of late complications due to involvement of the aorta, the arteries of the upper and lower limbs, or of other districts. (3-7) In cardiology, Horton disease is rarely encountered and is, consequently, underestimated.
We report four cases of GCA involving the aortic root and causing severe aortic incompetence in elderly women.
A 65-year-old woman was an inpatient in a cardiology center following paroxysmal episodes of nocturnal dyspnea and atrial tachycardia. Five years previously, she had been admitted to a department of internal medicine where the diagnosis of Horton disease had been made on the basis of the symptomatology, laboratory test results, and the findings of a biopsy of the temporal artery. After prolonged cortisone therapy, the patient was considered to be cured. On admission to the cardiology center, physical findings indicated severe aortic valve insufficiency. A chest radiograph showed an aneurysm involving the ascending tract and the arch of the aorta, and left ventricle enlargement. A two-dimensional echocardiogram and an angiogram confirmed these findings and also showed aortic valve insufficiency; the coronary arteries were unaffected. The results of laboratory tests were within normal limits. The patient underwent aortic valve replacement with implantation of a woven Dacron graft in the aneurysmal portion of the ascending aorta. Histologic examination of the aortic wall indicated giant cell aortitis. She was treated with steroids for 6 months, and she is doing well 3 years after surgery.
A 70-year-old woman entered a cardiac surgery unit for preoperative evaluation because over the previous 6 years she had developed an aortic ectasia with progressive valvular incompetence. Eight years before hospital admission, she had experienced polymyalgia rheumatica. As a result, she had been given corticosteroid therapy continuously for the first 2 years and then in several courses until she entered the cardiac unit. Despite a decrease in ESR and overall improvement, she never became totally asymptomatic. As there was no clinical evidence of temporal arteritis, a biopsy was not performed. She also had a history of COPD, and 5 months before hospital admission she had had an episode of heart failure. In the cardiac unit, an ECG revealed sinus rhythm and left ventricular hypertrophy. A color Doppler echocardiogram showed marked dilatation of the ascending aorta and the aortic arch with severe valve insufficiency. Valvular leaflets appeared to be quite normal. A CT scan of the chest and aortography confirmed the presence of a large aortic aneurysm with dilatation of the innominate artery (Fig 1). Coronary arteriography gave normal findings. The aortic valve and ascending aorta were replaced with a composite graft (ie, a Bentall operation). Pathologic examination of the aortic wall revealed GCA. On the 10th day after surgery, the patient died of multiorgan failure.
[FIGURE 1 OMITTED]
A 78-year-old woman with hypertension, in whom dilatation of the aortic root and moderate valve insufficiency had been diagnosed 3 years previously, was referred to our hospital because of a sudden episode of chest pain radiating to the back that was associated with dyspnea. An echocardiogram suggested aortic dissection. An aortogram showed a massive aneurysm of the ascending aorta with diffuse dilatation of the innominate artery, the aortic arch, and the descending aorta (Fig 2). There was severe valvular regurgitation; the coronary arteries were normal. The patient underwent the Bentall surgical procedure. Histologic examination of the aortic wall demonstrated giant cell aortitis. Laboratory tests, which were reconsidered following histology, indicated an increase in inflammatory indexes. A serum antineutrophil cytoplasmic antibody titer was negative. The patient is well 14 months following surgery.
[FIGURE 2 OMITTED]
A 75-year-old woman with a 5-year history of hypertension and exertional dyspnea was admitted to the hospital because of a sudden episode of respiratory distress. Physical examination revealed a BP of 160/70 mm Hg in both arms and a 3/6 grade aortic insufficiency murmur. An ECG showed left ventricular hypertrophy with ST-T changes. An echocardiogram disclosed a prominent dilatation of the ascending aorta extending to the innominate artery and severe aortic valve regurgitation with an apparently normal valve. These findings were confirmed by angiography, which also showed a coronary bed with no remarkable atherosclerotic lesions. ESR was 70 mm/h, and serum [[alpha].sub.1], fibrinogen, and C-reactive protein levels were elevated. The patient underwent a Bentall operation (Fig 3). A histologic diagnosis of giant cell aortitis was made. She is asymptomatic 10 months after surgery.
[FIGURE 3 OMITTED]
The first case refers to a woman showing typical features of Horton temporal arteritis. Despite prolonged steroid treatment, she developed severe aortic regurgitation due to giant cell aortitis over a 5-year period. In case 2, aortic ectasia occurred 2 years after the diagnosis of polymyalgia rheumatica, with local manifestations of temporal arteritis being absent. The other two cases involved women in whom the involvement of the ascending aorta was the first sign of Horton disease. In all cases, angiography showed no significant atherosclerotic lesions of the coronary arteries. A two-dimensional echocardiogram also disclosed an aortic valve that was regular in appearance. At the time of surgery, laboratory test results were in the normal range in case 1, while cases 3 and 4 were clearly indicative of an inflammatory status. In case 2, inflammatory indexes were normal on hospital admission. The patient, however, was still being treated with cortisone. The presence of active aortitis could be suspected in cases 1 and 2, while it was entirely unexpected in the others. However, histologic confirmation is needed in making a definitive diagnosis. The microscopic picture, almost identical in all cases, showed large areas of medial eosinophilic necrosis surrounded by a chronic inflammatory infiltrate with clusters of multinucleated giant cells. Areas of medial fibrosis and diffuse intimal thickening with scattered atherosclerotic lesions also were noted (Fig 4). Elastic van Gieson staining demonstrated severe, irregular loss of the elastic component (Fig 5).
[FIGURES 4-5 OMITTED]
Owing to the advanced age of the patients and the mild dystrophic changes of the aortic cusps, a Bentall operation was the treatment of choice. Because of the apparent integrity of the aortic sinuses in case 1, the ascending aorta and valve were separately replaced. In the other three patients, a composite graft with reimplantation of the coronary arteries was used. Alternative tissue valve conduits such as a homograft or a porcine aortic root probably would have been a better option for the patients but were not available in our institution at the time of surgery.
In case 2, the patient died soon after surgery because of multiorgan failure. The three other women were discharged after an uneventful recovery and were followed-up for a period ranging from 10 to 36 months (mean, 20 months). Only case 1 was given steroid therapy for 6 months after surgical treatment. The three women did not experience new episodes of arteritis and, at present, are in good health, with laboratory test results within the normal range.
Retrospective studies (7,8) show that GCA affects the aorta and its major branches in 15% of cases. In 75 to 83% of these patients, symptoms referable to large artery involvement usually occur 2 to 7 years after diagnosis, whereas in the remaining 17 to 25%, they are the presenting complaints of the disease (8,9)
Our first and second observations prove that an apparently successful treatment of the initial illness with high-dose steroid therapy does not invariably lead to complete histologic resolution of the arteritis. Our third and fourth cases suggest that, with the aneurysmal enlargement of the ascending aorta in elderly patients, biochemical parameters indicating an inflammatory process could bear out the diagnostic hypothesis of GCA, which might benefit from steroid therapy before surgery. (10)
However, patients with a typical history of Horton's disease and/or polymyalgia rheumatica should be closely followed-up by cardiologists to ensure the early detection of large-vessel pathology, which could be responsible for life-threatening complications such as aortic dissection and/or rupture, severe aortic incompetence, and ischemic syndrome of the upper and lower extremities. (2,4,6,7,10,11) Moreover, when cardiologists are faced with an aortic aneurysm in older individuals, particularly women, Horton disease should at least be suspected and other elements sought in the patient's clinical history in order to ascertain the nature of the aortopathy. An investigation of the superficial temporal arteries by duplex ultrasonography may also be of value. (12)
As for the histologic diagnosis of giant cell aortitis, an examination of several sections of the surgical specimen is mandatory. In fact, vasculitis is a dynamic process involving segmental areas of the arterial wall, and lesions are at different stages of evolution, some of which have little significance for diagnostic purposes. Furthermore, the overlap of atherosclerosis with GCA may render the evaluation of both angiographic and pathologic findings more difficult.
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* From the Department of Pathology (Drs. Nesi, Anichini, Pedemonte, Tozzini, and Gori), University of Florence, Florence, Italy; and the Cardiac Surgery Unit (Drs. Calamai and Montesi), Careggi Hospital, Florence, Italy.
Received March 8, 2001; revision accepted September 26, 2001.
Correspondence to: Franca Gori, MD, Department of Pathology, University of Florence, Via le Morgagni, 85, 50134 Florence, Italy
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