 Abstract | | |
Takayasu arteritis (TA) is an inflammatory process frequently associated with stenosis and obliteration of the aorta and its primary branches. We report a 16-year-old girl in whom TA manifested mainly with a sudden decrease in visual acuity and severe arterial hypertension in both legs. Bilateral radial pulses were absent. Ultrasound showed renal size asymmetry and raised the possibility of renal artery stenosis. The diagnosis of TA was confirmed by computed tomography angiography, which showed a thickened abdominal aortic wall and narrowing of its lumen. In addition, occlusions of left renal artery and of both left and right subclavian arteries were observed. Hypertension was hardly under control by a combination of three antihypertensive drugs. The outcome was favorable with corticosteroids alone with regression of the clinical signs, disappearance of inflammation, and control of hypertension.
How to cite this article:
Hassani M. Takayasu arteritis associated with severe renovascular hypertension. Saudi J Kidney Dis Transpl 2016;27:402-6 |
| Introduction | |  |
Takayasu arteritis (TA), also known as aortoarteritis and pulseless disease, is a rare condition. It is a chronic granulomatous vasculitis of unknown cause and affects large-and medium-sized arteries, primarily the aorta and its main branches. It can lead to progressive stenosis, occlusion, or aneurismal transformation. [1],[2] Clinically, TA includes an early "pre-pulseless" systemic phase with nonspecific symptoms of fever, night sweats, weight loss, arthralgia, and myalgia. [2],[3] As large-vessel inflammation progresses, more characteristic features become apparent due to wall thickening and narrowing, fibrosis, stenosis, and occlusion of the affected arteries. Diagnosis is based on the combination of suggestive clinical picture, increased levels of inflammatory markers, and diagnostic imaging. Because early symptoms often are nonspecific, prompt diagnosis of TA often is challenging.
We report an emergency presentation of TA with renovascular hypertensive crisis in a young girl.
| Case Report | | |
A 16-year-old girl with a six-month history of weakness and fatigability of the upper extremities, claudication of the lower limbs and diffuse arthralgia, presented to the emergency department after sudden decrease in visual acuity. Physical examination revealed high blood pressure (240/130 mm Hg) in both legs. There were no signs of volume overload manifested by clear chest and absence of peripheral edema. Renal bruits were not audible. The pulses in bilateral dorsalis pedis and posterior tibial arteries were weak, and bilateral radial pulses were absent. Fundoscopic examination showed papillary edema. The neurological examination was unremarkable. The family history was negative for essential hypertension and cardiovascular diseases.
Laboratory investigations revealed C-reactive protein (CRP) level: 1.7 mg/dL (reference range, <0.5 mg/dL), erythrocyte sedimentation rate (ESR): 42 mm/h, serum creatinine level: 0.5 mg/dL (44 μmol/L), leukocyte count: 10.4 × 10 3 /μL, urinalysis: moderate proteinuria, without hematuria or pyuria, and antinuclear antibody and anti-double-stranded DNA: Negative. Cerebral computed tomography (CT) angiography was normal. A diagnosis of hypertensive crisis associated with sudden decrease in visual acuity was made.
Ultrasound showed a small left kidney measuring 7 cm in the longitudinal axis with decreased parenchymal thickness and increased echodensity. The right kidney was increased in size (13 cm) with normal echodensity. Because of renal size asymmetry, the possibility of renal artery stenosis was raised.
Color Doppler indicated turbulent flow and spectral analysis suggested high peak velocity in the left renal artery and a decreased resistive index in the interlobular arteries, consistent with high-grade stenosis. The resistive index obtained in the right interlobular arteries was normal. Thoracoabdominal CT angiography showed occlusion of both subclavian arteries [Figure 1], occlusion of left renal artery, and thickening and narrowing of the sub-renal abdominal aortic wall, indicating an inflammatory process, such as aortitis [Figure 2] and [Figure 3]. Infectious causes of vasculitis were excluded. Serologic tests for Borrelia species, hepatitis B, and syphilis were performed with negative results. Polymerase chain reaction for mycobacterium tuberculosis was negative. Thus, the diagnosis of TA was made.  | Figure 1: Computed tomography angiography shows narrowing of left subclavian artery.
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 | Figure 2: Computed tomography angiography shows narrowing of the abdominal aorta and occlusion of left renal artery.
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 | Figure 3: Computed tomography scan shows circumscribed thickening of the abdominal aortic wall.
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The patient was then treated with oral prednisone (1 mg/kg/day). Hypertension was treated using three medications, namely perindopril 10 mg/day, nicardipin 50 mg twice daily, and acebutolol 200 mg/day. In the following days, the blood pressure values decreased, but did not reach normal values (150/95 mm Hg). We related this mild hypertension to the glucocorticoid treatment. CRP and ESR levels were normalized, and steroid dosage was tapered gradually to prednisone 5 mg/day after six months However, the absence of bilateral radial pulses persisted.
| Discussion | | |
The finding of hypertension and arterial bruits in young adults necessitates the examination of pulses and blood pressures in different limbs in order to detect asymmetry. Elevated ESR is a common finding; however, caution is advised, because up to 50% of patients may have active TA disease and a normal ESR. [4],[5],[6]
The diagnosis of TA is reached on the basis of clinical presentation and imaging results. Histopathologic confirmation can be obtained in patients who undergo vascular surgery.
At present, TA in children is diagnosed on the basis of the criteria proposed by European League Against Rheumatism Pediatric Rheumatology International Trials Organization Pediatric Rheumatology European Society, Ankara 2008, [7] presented in [Table 1].
Our index case met all the criteria for the diagnosis of TA. Considering the location of the arterial lesions, on the basis of angiographic findings, our patient was classified as TA of type III. [8]
In our patient, the initial inflammatory phase of TA did not occur. The onset of her disease was undetectable, and severe systolic arterial hypertension, which was revealed accidentally, was the predominant clinical manifestation.
The differential diagnosis can include certain congenital disorders that affect the tissue matrix (Marfan and Ehler-Danlos syndromes); however, these disorders are not associated with stenotic lesions in large vessels, which is the common finding in TA. Although infectious causes of large-vessel aneurysm (mycobacterial, syphilitic, or fungal) should be considered, these are similarly not associated with stenotic arterial lesions. Certain autoimmune illnesses (systemic lupus and Behçet's disease) can be associated with large-vessel vasculitis; however, these more typically have other distinguishing factors and distinct age predilection (Kawasaki disease and Giant cell arteritis). Perhaps, the most difficult distinction is between TA and giant cell arteritis. Both conditions involve large arteries, which show granulomatous vasculitis on histologic examination, and both respond to corticosteroids. Sarcoidosis should also be included in the differential diagnosis of TA. The correct diagnosis usually depends upon the presence of other characteristic features.
The mainstay of therapy in TA is immunosuppression, primarily with steroids or methotrexate. [9],[10] Alternative therapies such as azathioprine, cyclophosphamide, mycophenolate mofetil, and tacrolimus hydrate are also used in TA, especially for corticosteroid-resistant disease. [11] Hypertension should be treated aggressively often with multidrug regimen, but pediatricians should be warned against ACE inhibitors until renal artery stenosis has been excluded. Remission occurs in 40-60% and resistance to any therapy in 20% of the patients. Surgery is required in 30% of the patients; if possible, surgery should be delayed until the acute phase of the disease has passed.
TA is a chronic, progressive disease. Its degree of activity varies over time; the intensity of its inflammatory processes typically fluctuates between exacerbation and reduction or remission. Vascular involvement tends to be progressive and vascular complications of the cardiac, renal, and central nervous systems are the major causes of morbidity and death in TA, which is usually fatal when it remains untreated. Remission remains the goal of therapy. Identifying the comorbid complications and initiating aggressive treatment may afford better chances of symptom-free survival. Five year survival rates of 80-90% have been reported. [12],[13] Poor outcome depends on the presence of such complications as hypertension, aortic regurgitation, and aneurysm, and on the rate of progression of the disease. In our case, the outcome was favorable with corticosteroids manifested with the regression of clinical signs and the disappearance of inflammation as well as control of hypertension.
TA can cause an emergency hypertensive crisis with inflammatory renal artery stenosis causing severe renovascular hypertension. A prompt therapeutic approach with glucocorticoids and other immunosuppressive treatment can achieve full remission.
Conflict of interest: None declared.
| References | | |
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Correspondence Address:
Mohamed Hassani
Department of Nephrology and Hemodialysis, Military Hospital Moulay Ismail, Meknes
Morocco
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DOI: 10.4103/1319-2442.178581 PMID: 26997400 
[Figure 1], [Figure 2], [Figure 3]
[Table 1] |
