|Year : 2018 | Volume
| Issue : 1 | Page : 67-70
Takayasu arteritis in Umuahia, South East Nigeria
Ogba Joseph Ukpabi1, Abali Chuku2, Eme Mark Offia1, Osita Ikenna Okoli3, Uchechukwu Callistus Ibewuike4
1 Department of Internal Medicine, Division of Cardiology, Federal Medical Centre, Umuahia, Abia State, Nigeria
2 Department of Ophthalmology, Federal Medical Centre, Umuahia, Abia State, Nigeria
3 Department of Internal Medicine, Federal Medical Centre, Umuahia, Abia State, Nigeria
4 Department of Radiation Medicine, University of Teaching Hospital, Ituku Ozalla, Enugu State, Nigeria
|Date of Web Publication||7-May-2018|
Dr. Ogba Joseph Ukpabi
Department of Internal Medicine, Division of Cardiology, Federal Medical Centre, Umuahia, Abia State
Source of Support: None, Conflict of Interest: None
Takayasu arteritis is a rare, systemic inflammatory large vessel vasculitis of unknown etiology that predominantly affects women of childbearing age. It is defined as granulomatous inflammation of the aorta and its major branches. Although the disease has a worldwide distribution, it is observed more frequently in Asian countries. It is rare in the black race as only a few cases have been documented. The objective of this study is to present a case of Takayasu arteritis diagnosed in South East, Nigeria, which is a rare disease in blacks. A 34-year-old female with clinical and radiological features of Takayasu arteritis is presented and related literatures reviewed. Although Takayasu arteritis is rare in the black race, with heightened clinical suspicion and appropriate investigations, more cases may be diagnosed.
Keywords: Ocular ischemia, Takayasu, vasculitis
|How to cite this article:|
Ukpabi OJ, Chuku A, Offia EM, Okoli OI, Ibewuike UC. Takayasu arteritis in Umuahia, South East Nigeria. Nig J Cardiol 2018;15:67-70
| Introduction|| |
Takayasu disease (also known as pulseless disease) was first described by a Japanese ophthalmologist, Mikito Takayasu, in 1908 at a clinical meeting following the finding of some ocular lesions in a patient. Two Japanese physicians at the same meeting also reported similar eye findings in individuals whose wrist pulses were absent.
Takayasu arteritis is a rare, systemic inflammatory large vessel vasculitis of unknown etiology that most commonly affects women of childbearing age. It is defined as granulomatous inflammation of the aorta and its major branches.
The worldwide incidence of Takayasu disease is estimated at 2.6 cases/million/year. Although the disease has a worldwide distribution, it is observed more frequently in Asian countries. Takayasu disease is rare in the black race : underdiagnosis of the disease might have accounted for this rarity. Ogunbiyi and Falase had reported four cases, all women in South West of Nigeria. In 2003, Okeahialam et al. also reported a case in the northern part of Nigeria. None was seen in the literature for South East Nigeria. Oguntona in 2010 reported a case of Takayasu disease in a young black boy in Durban, South Africa.
Approximately 80% of patients with Takayasu disease are women. Most patients with Takayasu arteritis are aged 4–63 years with the mean age of onset being approximately 30 years.
Takayasu arteritis is an inflammatory disease of large- and medium-sized arteries with a predilection for the aorta and its branches. There is panarteritis with intimal proliferation in advanced lesions. Lesions produced by the inflammatory process can be stenotic, occlusive, or aneurysmal. Brachiocephalic or subclavian stenosis may result to subclavian steal syndrome. This arises from reversal of blood flow in the vertebral artery to the arm, thereby producing vertebrobasilar insufficiency.
The etiology of Takayasu arteritis is unknown. The underlying pathologic process is inflammation. Several etiologic factors have been proposed which includes spirochetes, Mycobacterium tuberculosis, and streptococcal organisms. Genetic factors may play a role in the pathogenesis.
The presentation of Takayasu disease is heterogeneous. Approximately 10% of patients with Takayasu arteritis are asymptomatic with the disease detected based on abnormal vascular findings on examination. Constitutional symptoms may precede clinical vascular involvement. Constitutional symptoms include headache, malaise, arthralgias, fever, and weight loss. Cardiac and vascular features include bruit, blood pressure (BP) difference at extremities, claudication, carotidynia or vessel tenderness, hypertension, renal artery stenosis, aortic regurgitation, and Raynaud's phenomenon. Pericarditis, congestive cardiac failure, and myocardial infarction may occur. Neurologic features include headache, visual disturbances, stroke, transient ischemic attack, and seizures, while dermatologic features are erythema nodosum, ulcerated subacute nodular lesions, and pyoderma gangrenosum.
Important differential diagnostic considerations include other large vessel vasculitides and collagen vascular diseases. Treatment of Takayasu disease is multidisciplinary. The mainstay of medical treatment is controlling the inflammatory process.
| Case Report|| |
The patient is a 34-year-old single female secretary from Abia State, South East Nigeria. She presented to us on February 14, 2014, with a 2-year history of visual impairment and 5-month history of fainting spells and headaches. There were associated progressive weight loss and fatigue, cessation of menstruation, and a pelvic swelling which were managed by the gynecology team as uterine fibroid.
She was first diagnosed hypertensive on presentation. The patient had no clinical symptoms suggestive of heart failure, coronary artery disease, or chronic kidney disease. There was no history of skin rashes, arthralgias, joint swelling, or night sweats. She is the first child in family of seven children. Parents and siblings are alive, and there was no family history of similar illness or hypertension.
Examination revealed a young woman that was afebrile (36.8°C), anicteric, pale with left eye redness, acyanotic, not dehydrated, no peripheral lymphadenopathy, no digital clubbing, and no leg edema. There were absent radial and brachial pulses bilaterally. Carotid, popliteal, posterior tibial, and dorsalis pedis artery pulsations were palpable bilaterally. Carotid pulse rate was 116/min, regular with bilateral carotid and supraclavicular bruits. BP measurements over the popliteal were 180/90 mmHg (R) and 190/100 mmHg (L). Jugular venous pressure was not raised. Apex beat was displaced. She had normal heart sounds with no murmurs. There were no significant findings on respiratory, central nervous, and digestive system examination.
Ocular examination findings showed significant pathology as shown in [Table 1].
The impression of Takayasu disease was made on clinical grounds which was later confirmed with computed tomographic (CT) angiography and color Doppler ultrasound of the aorta and its branches. CT thoracic angiography [Figure 1] showed occlusion of the brachiocephalic, left common carotid, and left subclavian arteries. Color Doppler ultrasound [Figure 2] also showed occlusion of brachiocephalic trunk, (L) common carotid, and (L) subclavian arteries. Echocardiography showed concentric left ventricular hypertrophy with mild pericardial effusion (0.9 cm) but good systolic and diastolic functions. Electrocardiography showed electrical left ventricular hypertrophy, chest radiography showed cardiomegaly with aortic unfolding, while abdominal ultrasound revealed a uterine fibroid. Erythrocyte sedimentation rate (ESR) was raised (84 mm/1 h), but fasting blood sugar, fasting lipid profile, full blood count, serum electrolytes, urea and creatinine, HIV testing, urinalysis, and stool microscopy were all normal. Pregnancy test, antineutrophil cytoplasmic antibodies (ANCAs), and antinuclear antibodies (ANAs) were negative.
|Figure 1: Computed tomographic aortic angiography of the index patient showing occlusion of the great vessels|
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|Figure 2: Color Doppler ultrasound of the brachiocephalic artery of the index patient showing absent flow of blood in BCA. BCA – Brachiocephalic artery; BCV – Brachiocephalic vein|
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The patient was commenced on tablets prednisolone 25 mg bd, tablets nifedipine 20 mg tds, tablets hydrochlorothiazide 12.5 mg daily, tablets aspirin 75 mg daily, gutt diclofenac tds, tablets indomethacin 25 mg bd, tablets omeprazole 20 mg bd, and other supportive treatment. She made a significant general improvement on treatment. Fatigue and headaches subsided, menstruation recommenced, and BP was controlled; she was discharged to the outpatient clinic. She is keeping her appointments and has remained on clinical remission.
| Discussion|| |
Takayasu arteritis is rare among blacks.,, The few cases documented in Nigeria and the one being presented here are mostly women  which is in keeping with the worldwide female predominance in Takayasu arteritis prevalence. With heightened clinical suspicion and appropriate investigations, more cases may be diagnosed among blacks. The features of Takayasu arteritis may be seen in other large vessel vasculitides and other collagen vascular diseases and hence the need to follow an internationally recognized criteria for diagnosis. In the American College of Rheumatology (ACR) criteria, three or more criteria yield a sensitivity of 90.5% and a specificity of 97.8%. The criteria are as follows:
- Age of 40 years or younger at disease onset
- Claudication of the extremities
- Decreased pulsation of one or both brachial arteries
- Difference of at least 10 mmHg in systolic BP between arms
- Bruit over one or both subclavian arteries or abdominal aorta
- Arteriographic narrowing or occlusion of the entire aorta, its primary branches or large arteries in the upper or lower extremities that are not due to arteriosclerosis, fibromuscular dysplasia, or other causes.
The index patient is a 34-year-old female, who presented with absent brachial and radial pulses bilaterally and a difference of 10 mmHg in systolic BP between the two lower limbs (measured over the popliteal artery as the Korotkoff sounds were absent over the brachial artery). There were bilateral carotid and supraclavicular bruits. Both the CT thoracic angiography and color Doppler ultrasound of the aorta of the index patient showed occlusion of the branches of the aortic arch (brachiocephalic, left subclavian, and left common carotid arteries).
The index patient meets the criteria for the ACR diagnosis of Takayasu arteritis.
The ocular pathology seen in this patient is a common presentation in Takayasu arteritis.,, Hypertensive retinopathy and ocular ischemia as seen in the index case are common pathogenetic mechanisms of ocular involvement. Arteritis involving the aortic arch and its branches favors the development of ischemic ocular complications. The patient presented with severe hypertension and hypertension is seen in 50% of cases of Takayasu arteritis. Hypertension in this patient may have been caused by chronically damaged rigid aorta. The more common explanation for hypertension in Takayasu arteritis is stenosis of the renal arteries, but this was not revealed by the patient's aortogram. Hypertension may be responsible for the left ventricular hypertrophy seen in this patient. Headaches and fainting spells may have been due to stenosis of the carotid arteries or subclavian steal phenomenon. Elevated ESR is in keeping with an inflammatory process and can be seen in some other inflammatory diseases such as giant-cell arteritis, spondyloarthropathies, systemic lupus erythematosus, rheumatoid arthritis, and Wegener's granulomatosis, but their other characteristic clinical features and negative ANA and ANCA tests make these differentials less likely.
The patient responded well to oral prednisolone, and there has been no relapse of active inflammation since commencement of oral steroids. Steroids are the mainstay of medical treatment. Other therapeutic agents that may play a role include interleukin-6 receptor inhibitors, for example, tocilizumab, B-cell depletion using anti-CD20, for example, rituximab, cytotoxic drugs for steroid-resistant cases or cases that relapse for example methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil, and tacrolimus,, and antitumor necrosis factor inhibitors. Cardiovascular procedures such as bypass graft surgery, percutaneous balloon angioplasty, angioplasty and stenting, aneurysm clipping, and revascularization are advanced options. Antihypertensive therapy, dyslipidemia treatment, antiplatelet and anticoagulation therapies, and lifestyle modification are adjuvant therapies.
| Conclusion|| |
We have presented a young female in South East Nigeria with Takayasu arteritis, a rare vasculitis in blacks. With heightened clinical suspicion and appropriate investigations, more cases may be diagnosed.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]