Complex supraaortic lesions in Takayasu arteritis: how to treat them?

THERAPEUTIC CHALLENGE

Complex supraaortic lesions in Takayasu arteritis: how to treat them?

Ricardo de Alvarenga Yoshida^I; Winston Bonetti Yoshida^II; Ralf Kolvenbach^III; Marcelo Hirga^IV; Paulo Roberto Bahdur Vieira^V

^IDoutorando. Colaborador, Disciplina de Angiologia, Cirurgia Vascular e Endovascular, Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, SP. Cirurgião vascular e endovascular. Cirurgião endovascular, Angiocenter, São José dos Campos, SP

^IILivre-docente. Professor adjunto, Disciplina de Angiologia, Cirurgia Vascular e Endovascular, Faculdade de Medicina de Botucatu, UNESP, Botucatu, SP

^IIIProfessor titular e chefe, Serviço de Cirurgia Vascular e Endovascular, Augusta Hospital, Dusseldorf, Alemanha

^IVCardiologista intervencionista, Angiocenter, São José dos Campos, SP

^VCirurgião vascular e endovascular e chefe, Serviço de Cirurgia Vascular, Santa Casa de São José dos Campos, São José dos Campos, SP.

Correspondence

Keywords: Takayasu’s arteritis, angioplasty, carotid artery diseases.

Introduction

Takayasu arteritis (TA) is a progressive chronic inflammatory disease characterized by being a systemic vasculitis. Such disease presents transmural granulomatous inflammation with intimal and adventitial fibrosis, as well as degeneration and disintegration of the medial layer,¹ causing stenosis, occlusions, dilatations and/or formation of aneurisms mainly in the supraaortic arteries,² with the possibility of originating neurologic symptoms (syncope, headache, stroke) and functional disability of the upper limbs.^2-4 Complementary tests are not of much help for its diagnosis.^2,4

The incidence of the disease is higher in young and Asian women. The prevalence reported is of 1.2, 2.6 and 2.9 million/year in the USA,^2,5 in Europe,² and in Mexico,^2,6 respectively. The incidence in Asia is 100 times higher.^2,3,5,7-9

Drug treatment with steroids and immunosuppressants is very important, mainly in the initial and acute phases of the disease. Conventional and endovascular surgical treatments are restricted to situations where there is impaired blood flow or functional disability of the affected limbs.¹ Some cases require combined clinical and surgical treatments.¹ Nevertheless, conventional and endovascular surgical treatments are not free of acute and late complications.^1,9-11

The objective of this therapeutic challenge is to discuss the difficulties of choosing the best treatment for a case of severe and diffuse cerebrovascular TA.

Part I - Clinical case

Female Caucasian patient, aged 28 years, who worked as a typewriter, presented with intense, unilateral headache in April 2008. Headache did not resolve with common analgesics and it was followed by discomfort, nauseas, and vomiting. The patient sought medical care at an emergence room (ER), where she received medication and was discharged after improvement of the symptoms. However, the patient had several relapses of the symptoms, even when being on medication until she was admitted for diagnostic investigation and analgesia.

Upon hospital admission, the patient complained of intermittent episodes of dizziness, faint, and mild bilateral amaurosis. She was seen by a neurologist who suspected of stroke. Cranial tomography evidenced stroke in the left occipital lobe and in the transition zone of the vascular regions of the anterior and medial cerebral arteries to the left and in the frontoparietal region and to the right in the posterior parietal region. Next, a magnetic resonance imaging (MRI) was carried out and it also showed stroke in the same regions. The patient also underwent Doppler ultrasound of the carotid and vertebral arteries (Figure 1) and arteriography, both showing occlusion of the left common and internal carotid arteries, segmental occlusion of the left prevertebral subclavian artery and critical stenosis (> 70%) of the right internal carotid artery (Figure 2). In spite of the severe obstructive lesions in the upper limbs, the patient had only a discrete claudication in the left upper limb.

The results of blood test, renal and hepatic function tests and serum levels of lipids were within normal limits. On the other hand, erythrocyte sedimentation rate (ESR) and C-reactive protein were high (118 mm and 16.2 g/dL) compared to the reference values (40 mm and 2.0 g/dL), respectively, suggesting a diagnostic hypothesis of TA based on the clinical and laboratory criteria and considering that the patient had met all clinical and angiographic criteria of the American College of Rheumatology,^11,12 as well as the Ishikawa criteria⁸: the obligatory criterion, one major criterion and two minor criteria.

The patient was treated with antiplatelet drug (ASA), corticosteroid therapy with prednisone (including pulse therapy) and cyclophosphamide with the purpose of reducing the inflammatory activity of the disease. The doses of corticosteroids and immunosuppressants varied from one week to the other based on the rheumatologist's decision. There was complete remission of the stroke symptoms after 20 days of treatment, but the patient stayed in hospital for 45 days until the inflammatory activity was reduced. After 4 months, the ESR and the C-reactive protein level decreased to 60 mm and 6.2 g/dL (reference values 40 mm and 2.0 g/dL), respectively. However, she had another transient ischemic episode in the contralateral body half, characterized by mild amaurosis.

Due to the worsening of her state and the risk of another stroke, some therapeutic possibilities were considered, namely:

• to maintain only the clinical treatment;

• to carry out a surgical angioplasty of the right carotid artery, beginning in its bifurcation and extending to the right internal carotid artery using a patch;

• to perform an angioplasty using a balloon catheter in the right internal carotid artery;

• to perform an angioplasty with placement of stent in the right internal carotid artery.

Part II - What was done

The medical team decided for the surgical treatment with the purpose of restoring the cerebral vessels. Endovascular treatment was chosen because it is less invasive, the patient was young, had a short neck, which was altered due to the long treatment with corticoids, and had weak immune defense caused by corticosteroid therapy and chemotherapy. Surgery preparation included cardiologic evaluation and antiplatelet drug treatment combined with clopidogrel.

The surgery was performed in the hemodynamics and endovascular surgery room of Angiocenter, São José dos Campos (SP). The patient received general anesthesia. Femoral access was conducted and a 6F x 90-cm Brite Tip guiding catheter (Cordis^®) was inserted with the help of a 0.035" x 260-cm hydrophilic stiff Road-Runner wire guide (Cook^®). Under systemic heparinization (10,000 U of unfractioned heparin intravenously), an angiography of the brachiocephalic artery and right common carotid artery was performed using a 5F x 100-cm vertebral catheter. The same wire guide was inserted in the right common carotid artery below the stenosis, and the guiding catheter was pushed forward until the medial segment of the right common carotid artery. Using Road-map viewing, the cerebral embolic protection device Interceptor (Medtronic^®) was inserted and deployed in a straight segment of the right internal carotid artery above the stenosis. A successful angioplasty of the stenotic lesion was carried out using a 6 x 20-mm Amia balloon catheter (Cordis^®), but there was wall dissection, evidenced as a flap in the angiographic control (Figure 3). Then, a 7 x 30-mm Exponent self-expandable stent (Medtronic^®) was placed, covering the dissection area (Figure 4).

The patient evolved well without neurologic or hemodynamic complications. On the second postoperative (PO) day, she had intense headache, which was refractory to common analgesics. The neurologist indicated computed tomographic (CT) scan, which showed normal results, suggesting a diagnosis of hyperperfusion syndrome. Since this state persisted, a new CT scan was performed on the third PO day, which showed a small ischemic area in the cortico-subcortical region of the right parietal lobe, with a mild expansive effect characterized by deletion of the adjacent cortical grooves and clefts. However, the patient did not present with motor and sensitivity deficits. Headache resolved spontaneously on the fourth PO day, and the patient was discharged from hospital on the fifth PO day.

A follow-up ultrasound examination of the carotid artery was carried out 3 and 6 months after the surgery and showed that the stent was well located, with peak systolic and final diastolic velocities within normal limits (Figure 5). The patient did not show any motor or sensitivity deficits.

Discussion

The etiology of TA remains unclear. Allergic and self-immune responses to previous infections have been related to the triggering mechanisms of this disease.^1,11 Several studies have demonstrated the relationship between the HLA genes and TA, suggesting a genetic susceptibility to the disease.^1,13

The chronic characteristic of this disease, with gradual development of collateral blood flow and tissue adaptation to poor perfusion, may explain the fact that patients with severe impairment of extracranial brain blood flow remain alive even with a low quality of life.¹ In the present case report, we found diffuse impairment of TA in the extracranial supraaortic vascular region; although being symptomatic, it could have caused more severe sequelae than those we found.

Clinical treatment should be the first option, mainly with regards to the reduction in the inflammatory activity.^{1,2,9-11,14,15} There are promising results with the use of methotrexate,^3,12,14,15 azatioprin^14-17 and, mainly, interferon-gamma^13-15,18 and anti-TNF.^14,15,19,20 Nevertheless, some patients do not present remission of the symptoms or inflammatory state even when receiving the appropriate treatment. The progressive worsening of the symptoms may support surgical intervention.^4,9,10,14,15

For the treatment of stenoses and occlusions caused by this disease, conventional surgical treatment shows the best long-term results with a rate of restenosis/reocclusion between 20-30%.^{4,9,10,14,15,21} This treatment basically consists of saphenous vein bypass or prosthesis more often originated from the aortic arch.¹⁴ However, of 57% of patients who received bypasses or underwent surgical reconstructions, 36% had restenosis or occlusions.^1,10

After the recent advances of the endovascular therapy, this procedure started to be attractive for the treatment of lesions caused by TA.^22,23 Angioplasties with or without stent placement have been described to have good short-term results^{4,14,15,24-26}. Results seem to be better with simple balloon angioplasty without stenting.^2,14 Therefore, the angioplasty using a balloon catheter could be considered as the first-choice surgical treatment, since it is a simple, little invasive procedure, showing excellent results in the atherosclerotic disease; however, the relapse rate is usually high in TA cases.^{1,4,9,10,14,21,22,26} With regard to stenting, a rate of 78% of restenosis has been found in a review of patients in the USA. In this same review, the comparison with the other centers in the world showed that the intervention with simple balloon angioplasty or stenting ranged from 12 to 50% of the cases.^9,10 With regard to our patient, it was necessary to stent due to the wall dissection just after simple balloon angioplasty.

The benefits of surgical treatment are reported in about 88%¹ when used in the initial phase of the disease. The results of the conventional or endovascular surgical treatment are often better after remission of the inflammatory process,² which is not always possible. In the present case report, while waiting for the remission of the inflammatory phase with the use of clinical treatment, there was a new neurologic event, making it mandatory to perform the surgical intervention even with the C-reactive protein level still a little above normal (CRP = 6.0).

Diffuse arterial involvement in TA increases the risk of procedures and their possible complications such as significant local vascular reactions that may increase the risk for thrombosis.^1,9,14,15,22 Another extremely severe complication is the cerebral hyperperfusion syndrome (CHS),¹ which remains as an unsolved problem. Our patient had CHS, which was clinically treated with strict control of the systemic blood pressure and clinical observation, but fortunately it evolved well.

In conclusion, to choose between conventional or endovascular surgical indication is not an easy decision in such cases, also because the vascular damage caused by angioplasty in an artery undergoing an inflammatory process may stimulate the development of local thrombosis, with worsening of the patient's clinical status, in addition to the fact that this treatment is not definitive.¹ However, there are extremely severe situations, with risk of significant sequelae, for which the surgical treatment is mandatory. Such treatment should be individualized and it should be combined with the clinical treatment with the purpose of achieving better late results. Further studies involving better endovascular devices must be conducted in order to reduce the high rates of restenosis/reocclusion in this disease.

References

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Publication Dates

• Publication in this collection
  26 Mar 2010
• Date of issue
  Dec 2009