| Abstract|| |
Takayasu arteritis (TA) is a rare chronic granulomatous inflammatory arterial disease of unknown etiology that affects the aorta, its main branches and pulmonary artery. The clinical presentation is nonspecific, with signs and symptoms that vary according to the affected arterial segment. The most commonly affected vessel is the subclavian artery, while renal artery stenosis is relatively uncommon. We report a case of a 57-year-old male patient with late diagnosis of TA and various related complications including stroke in the left middle cerebral artery territory, predominant left renal artery stenosis, and hypertension with discrepancy of blood pressure between two arms due to predominant left subclavian artery stenosis. Thus, physicians should keep in their mind this late presentation after the age of 40 years. The aim is to increase the awareness of this condition because of early diagnosis and the timely introduction of treatment can lead to improved outcomes in this poorly understood clinical enigma.
|How to cite this article:|
ur Rahman SS, Al Shehri A, Al Ghamdi M, Al Shareef M. Stroke and renal artery stenosis from Takayasu’s arteritis diagnosed in a 57-year-old male patient. Saudi J Kidney Dis Transpl 2019;30:710-4
|How to cite this URL:|
ur Rahman SS, Al Shehri A, Al Ghamdi M, Al Shareef M. Stroke and renal artery stenosis from Takayasu’s arteritis diagnosed in a 57-year-old male patient. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2019 Jul 23];30:710-4. Available from: http://www.sjkdt.org/text.asp?2019/30/3/710/261353
| Introduction|| |
Takayasu arteritis (TA) also known as aorto arteritis and pulseless disease is a rare chronic granulomatous inflammatory arterial disease of unknown etiology; although, some studies have linked TA to human leukocyte antigen Class BW52 antigen, which is affecting the aorta and its main branches.,,,, It mainly affects women in their reproductive years and is more commonly seen in Southeast Asia.,,,
Clinically, TA includes an early “pre-pulseless” systemic phase with nonspecific symptoms and as large-vessel inflammation progresses it can lead to progressive stenosis, occlusion, or aneurysmal transformation. Diagnosis is based on the combination of the suggestive clinical picture, increased levels of inflammatory markers, and diagnostic imaging. The gold standard test is angiography in the diagnosis of TA; however, magnetic resonance angiography (MRA) and computed tomography angiography (CTA) are alternatives and can also detect vessel wall thickening; positron emission tomography scan can help to detect vessel wall inflammation.
We report a case of TA with stroke and renal artery stenosis in a 57-year-old male patient which is an unusually late presentation of TA; however, now, there is a high prevalence in the elderly based on some case reports.
| Case Report|| |
Informed consent was obtained from the patient before reporting the case.
A 57-year-old Saudi male patient from Taif with a history of active smoking and no regular medical follow-up in the past was admitted to the Security Forces Hospital in Makkah for the sudden onset of right-sided weakness and aphasia. CT of the brain showed left middle cerebral artery infarct outside window for tissue plasminogen activator and endovascular intervention.
His physical examination revealed aphasia, facial asymmetry, and right-sided weakness with power 0/5 and up going right plantar reflex. Notably, there was discrepancy of pulse and blood pressure between two arms with blood pressure 108/78 on the left side and 164/89 on the right side. He had weak femoral pulses bilaterally and bruit over the left subclavian, carotid, and renal arteries.
Initial laboratory investigation showed complete blood count, liver function tests, lipid profile, and blood sugar within the normal limits and serum creatinine of 1.4 mg/dL.
Electrocardiograph showed no ischemic changes, echocardiogram showed left ventricular hypertrophy and mild aortic regurgitation.
Renal ultrasound with Doppler studies showed small-sized left kidney measuring 7 cm in its longest axis and right kidney measured 10 cm. Resistive index of segmental arteries showed a difference of 0.10 between the right and left segmental arteries (right 0.75 and left 0.60), suggesting left-sided renal artery stenosis.
CTA of thoracoabdominal aorta showed patent aorta with diffuse irregular thickening of the whole length of its wall and discrete atheromatous mural calcifications. In addition, there was attenuated ostia of the left subclavian and left common carotid arteries [Figure 1], non-visualized ostia of the celiac trunk as well as superior mesenteric artery with dilated collaterals. The right common iliac artery was seen relatively attenuated at its origin. The left renal artery was seen markedly attenuated at its origin, otherwise of average caliber, showing subsequent left renal atrophic changes. The right renal artery showed relative attenuation at its origin, otherwise of average caliber [Figure 2].
|Figure 1: Computed tomographic angiography showing stenosis of the left subclavian artery.|
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|Figure 2: Computed tomographic angiography showing marked stenosis of the left renal artery with relatively small-sized kidney.|
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Magnetic resonance imaging of the brain showed extensive left fronto-parietal -anterior temporal subacute infarction [Figure 3].
|Figure 3: Magnetic resonance imaging of the brain showing extensive left fronto-parietal anterior temporal infarction.|
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MRA of the brain showed attenuation and irregularity of cavernous and supraclinoid left internal carotid artery and M1 and M2 segment of the left middle cerebral artery as well as beaded appearance of most of the terminal branches of both middle cerebral artery and posterior cerebral vessels, denoting vasculitis [Figure 4].
|Figure 4: Magnetic resonance angiography brain: Showing attenuation and irregularity of cavernous and supraclinoid left internal carotid artery and M1 and M2 segment of the left middle cerebral artery as well as the beaded appearance of most of the terminal branches of both middle cerebral and posterior cerebral vessels denoting vasculitis.|
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Other laboratory investigations showed negative antinuclear antibody, anti-neutrophilic cytoplasmic antibody, rheumatoid factor and serologies for hepatitis B, C and HIV. The erythrocyte sedimentation rate was 80 mm after the 1st hour and C-reactive protein was 2.8 mg/dL. The tuberculin test was negative.
Based on the above clinical picture and imaging findings, TA was diagnosed, and the patient was treated with pulse steroid (methyl-prednisone 1000 mg intravenously for three doses) followed by oral prednisone 1 mg/kg daily with azathioprine 100 mg orally daily and continued on aspirin, atorvastatin, and amlodipine with physical therapy. The patient had a slight improvement in right side weakness and blood pressure was well controlled with single antihypertensive medication. He was referred to vascular surgery for angio-plasty and possible surgical revascularization.
| Discussion|| |
Chronic and slow-developing TA causes vascular lesion characterized by thickening of the adventitia and cellular infiltration of the tunica media, with local destruction of smooth muscle cells and elastin of the vessels. The intimal hyperplasia results from the proliferation of myofibroblasts, followed by fibrosis of the media and intima, leading to stenosis, and occasionally overlapping atherosclerosis of the affected arterial segment.,
The diagnostic criteria for TA, defined by the American College of Rheumatology, include: age of onset <40 years, claudication of the limbs, decreased pulse of the brachial artery, difference in systolic blood pressure >10 mm Hg between the two arms, and murmurs of the subclavian arteries or the aorta and abnormal arteriography. Having three or more of these criteria yields a sensitivity of 90.5% and specificity of 97.8%.
Our index case met four criteria, with an unknown age of onset and late diagnosis. Considering the location of the arterial lesions on the basis of angiographic findings, our patient was classified as TA of type V.
The clinical expression is varied and may be asymptomatic for several years, or present as nonspecific systemic symptoms such as fever, asthenia, arthralgia, myalgia or night sweats, and more rarely, acute vascular events. The nonspecific nature of the symptoms on presentation, combined with the absence of physical symptoms, generally results in late diagnosis and failure to start proper treatment early in the course of the disease. According to Kerr et al, 20% of the patients are not diagnosed for three years after the onset of symptoms. This demonstrates the difficulty in diagnosis and corroborates the fact that several recent studies have shown a high prevalence in the elderly.
The subclavian arteries are by far the most common angiographic manifestations of TA (93%). Carotid and renal arteries are involved in 58% and 38% of patients, respectively. However, there are racial differences in the pattern of vessels affected by this disease with most Japanese patients having involvement of aortic arch and its branches and in Indian patients, predominantly the abdominal aorta and its branches are affected.
Renal artery stenosis is mostly caused by atherosclerosis or fibromuscular dysplasia, and hence, it is very rare to have this stenosis as a manifestation of systemic vasculitis., Nevertheless, some clinical and laboratory parameters such as pulseless extremities and elevated C-reactive protein levels may indicate systemic vasculitis and warrant further diagnostic procedures, since only an anti-inflammatory approach can avoid or at least reduce other complications of vasculitis. Usually, most studies on TA are focused on brachiocephalic involvement, with no reports on the exact consequence of renal stenosis, but it is known that it can result in hypertension and fatal cardiovascular events., In 33%–83% of cases of TA, there is arterial hypertension, and in 20%–38% of these patients, renal artery stenosis contributes to this factor. Other causes of hypertension are atypical coarctation, reduced aortic capacitance as well as diminished baroreceptor reactivity. However, hypertension can often be hidden, since the blood pressure measured in the upper limbs may underestimate the true central pressure as a result of the subclavian artery involvement.
Conditions that should be considered in the differential diagnosis of TA include giant cell arteritis, IgG4 related disease, Behcets syndrome, Cogan syndrome, infectious aortitis, fibromuscular dysplasia, atheriosclerosis, Ehlers-Danlos syndrome More Details, relapsing polchon-dritis, and spondylo-arthropathies. None of the above diseases fit the clinical scenario of our patient.
TA is a chronic, progressive disease. Its degree of activity varies over time; the intensity of its inflammatory processes generally fluctuates between exacerbation and reduction or remission. The poor outcome depends on the presence of complications such as stroke, hypertension, aortic regurgitation, aneurysm, and on the rate of progression of the disease. TA-related mortality rates have been described as >35% in five years.
In our case, the hypertension was well controlled after steroid use even with single antihypertensive medication, but patient was disabled due to massive cerebral ischemic infarct.
This arterial disease remains a clinical challenge at all stages and treatment decisions are hampered by a shortage of evidence pro and against targeted therapies. The difficulty in dealing with TA is a reflection of the nature of the disease and its rarity, which limits the feasibility of future clinical trials.
Conflict of interest: None declared.
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Sayed Shakeel ur Rahman
Security Forces Hospital Program, Makkah, Kingdom of Saudi Arabia
Kingdom of Saudi Arabia
[Figure 1], [Figure 2], [Figure 3], [Figure 4]