Aminde, Leopold N

Dzudie, Anastase

Njim, Tsi

Noubiap, Jean Jacques N.

Takah, Noah

English

PubMed

Takayasu arteritis is rare in black people. Doppler echocardiography may assist in its diagnosis with good response to steroids

Aminde, Leopold N.

Takah, Noah F.

University of Queensland eSpace

Takayasu’s arteritis in an adult female from Cameroon: diagnosis via Doppler echocardiography

 Lesson  Takayasu arteritis is rare in black people. Doppler echocardiography may assist in its diagnosis with good response to steroids. </jats:p

Leopold N Aminde

Jean Jacques N Noubiap

Noah Takah

Tsi Njim

Anastase Dzudie

Crossref

JRSM Open

Takayasu arteritis is rare in black people. Doppler echocardiography may assist in its diagnosis with good response to steroids.Full Tex

Noubiap, Jean Jacques N

Griffith Research Online

Takayasu's arteritis in an adult female from Cameroon: diagnosis via Doppler echocardiography.

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Takayasu's arteritis in an adult female from Cameroon: diagnosis via Doppler echocardiography

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Case ReportTakayasu’s arteritis in an adult female from Cameroon:diagnosis via Doppler echocardiographyLeopold N Aminde1,2, Jean Jacques N Noubiap3,4, Noah Takah5, Tsi Njim6 andAnastase Dzudie7,81Clinical Research Education, Networking and Consultancy, Douala, Cameroon2Faculty of Medicine & Biomedical Sciences, School of Public Health, The University of Queensland, Brisbane, Australia3Department of Medicine, Groote Schuur Hospital and University of Cape Town, Cape Town, South Africa4Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa5School of Medicine, University of Glasgow, UK6Regional Hospital Bamenda, Bamenda, Cameroon7Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa8Department of Internal Medicine, Faculty of Health Sciences, University of Buea and Cardiology Unit, General Hospital Douala,CameroonCorresponding author: Leopold N Aminde. Email: amindeln@gmail.comLessonTakayasu arteritis is rare in black people. Doppler echocar-diography may assist in its diagnosis with good response tosteroids.KeywordsTakayasu’s disease, pulselessness, arteritis, doppler echocar-diography, CameroonCase presentationA 30-year-old lady presented to our unit with a historyof progressive ipsilateral left upper and lower limbweakness on exertion for over four years. Thiswas asso-ciated with intermittent aches in aﬀected limbs whichworsened on exertion. She occasionally experiencedshortness of breath but no orthopnoea, chest pain,cough nor leg swelling. She consulted at several primarycare hospitals with no amelioration of symptoms.On examination, she was stable and not in any dis-tress, apyretic with absent pulses on left upper limb.There was no blood pressure reading on aﬀected limbbut the right arm had a blood pressure of 169/79mmHgand pulse of 69 beats per minute. There was a rightcarotid bruit and very faint left carotid pulse. The restof the peripheral pulses were normally palpable.Heart, neurological and the rest of the examinationwas unremarkable. Investigations included: a normalfull blood count; raised erythrocyte sedimentationrate: 25mm at ﬁrst hour (normal 10mm) and59mm at second hour (normal 25mm); normal C-reactive protein: 0.29mg/L (normal 5mg/L);normal serum creatinine: 7mg/L; negative hepatitisB and C serological tests; normal thyroid functiontests; negative anti-nuclear antibody test; and negativeVeneral Disease Research Laboratory and Treponemapallidum hemagluttin antigen tests . Chest X-ray, elec-trocardiography and cardiac echography were normal.Doppler vascular assessment revealed signiﬁcant reduc-tion of abdominal aortic lumen from inﬂammation,occlusion of left carotid, subclavian, axillary and verte-bral arteries. There was also involvement of the bra-chial, ophthalmic and radial arteries. A reverse ﬂowpattern was observed in the left common carotid, withperfusion of left upper limb from brain (Figures 1 to 5).With extensive vascular lesions, pulselessness, carotidbruit and blood pressure discrepancies, a diagnosis ofTakayasu’s arteritis (TA)was proposed. Shewas placedon steroids and at two months follow-up, reportedmarked amelioration of symptoms, but was later lostto follow-up.DiscussionTakayasu’s disease, variably known as ‘Takayasu’sarteritis’, ‘pulseless disease’, ‘occlusive thromboaorto-pathy’, ‘Martorell syndrome’ or ‘non-speciﬁc aorto-arteritis’, is a chronic vascular inﬂammatorycondition aﬀecting large arteries predominantly theaorta and its branches.1 The International ChapelHill Consensus conference of 2012 nomenclaturesystem divided vasculitides into large-vessel, medium-vessel and small vessel vasculitis; TA was grouped aslarge-vessel vasculitis together with giant cell arteritis.2A female (in their second and third decades) predom-inance has generally been recorded in a majority of thecases with female-to-male ratios as high as 9:1in Japan.3 In fact, in a majority of patients younger! 2016 The Author(s)Creative Commons CC-BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License (http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission providedthe original work is attributed as specified on the SAGE and Open Access page (https://uk.sagepub.com/en-us/nam/open-access-at-sage).Journal of the Royal Society of Medicine Open;7(8) 1–5DOI: 10.1177/2054270416654646 at Glasgow University Library on September 19, 2016shr.sagepub.c mDownloaded from than 50 years, TA has been shown to be the mostfrequent cause of systemic vasculitis aﬀecting theaorta.4 Despite limited epidemiological studies withscant reports from Africa, TA is relatively morecommon in Asia with estimated population prevalenceof 0.004% in Japan.5TA generally involves two stages: the pre-pulseless(pre-stenotic) phase, in which the patient hasnon-speciﬁc symptoms, followed by the pulseless(stenotic/occlusive) phase, in which there is signiﬁcantvascular insuﬃciency with diminished to impalpablepulses (commonly upper limb), and bruits may beheard over the aﬀected vessels.1 The distribution of vas-cular aﬀection of the disease also varies by region, withcervical and thoracic arterial lesions being morecommon in Japan and South America contrary toabdominal lesions in other Asian countries.5 The cor-onary ostia, pulmonary arteries and renal arteries mayalso be involved. Thus, clinical manifestations are vari-able, ranging from being asymptomatic to subclaviansteal syndrome and even cerebrovascular accidents.6The diagnosis of TA requires at least three of theAmerican College of Rheumatology criteria(Table 1)7 to be present. This criterion is said tohave a sensitivity of 91% and speciﬁcity of up to98%. Our patient fulﬁlled all criteria except arterio-graphic evidence. Magnetic resonance angiography,CT imaging and histology are generally required forconﬁrmation of the disease. Histology often showsgranulomatous inﬂammatory inﬁltrates with macro-phages, B- and T-lymphocytes, eosinophils andplasma cells with presence of giant cells. This granu-lomatous inﬂammation in vessel adventitia eventuallyprogresses to panarteritis.6 Blood tests also showraised inﬂammatory markers. In the late phases ofthe disease, four types have been described8; type 1– classic pulselessness involving brachiocephalictrunk, carotid and subclavian arteries; type 2 – acombination of type 1 and 3; type 3 – atypical coarc-tation involving thoracic and abdominal aortas distalto the arch and its major branches; type 4 – extensivedilation of the length of the aorta and its majorbranches. The most frequent of them is type 3 (65%of cases) commonly involving subclavian, leftcommon carotid, brachiocephalic trunk, renal,Figure 2. Doppler pulsed wave image showing luminal reduction and decreased flow velocity in axillary (left panel) and Brachial(right panel) arteries.Figure 1. Echographic image of the abdominal aortashowing reduction in lumen from inflammation.2 Journal of the Royal Society of Medicine Open 7(8) at Glasgow University Library on September 19, 2016shr.sagepub.comDownloaded from coeliac, superior mesenteric and pulmonary arteries.Quite rarely it may involve the axillary, brachial, ver-tebral, coronary and iliac arteries.8Recently, doppler echocardiography has beendemonstrated to be of high diagnostic use foreven early (pre-stenostic) TA.9 Schmidt et al.demonstrated good correlation of colour dopplersonography with angiographic features of patientswith non-speciﬁc symptoms and features of sys-temic inﬂammation. They suggested that colourdoppler imaging of primary extra cranial branchesof aortic arch (subclavian, carotid) could be verycrucial for the diagnosis of TA even in the pre-stenotic phase of the disease. The diﬀerential diag-noses for TA are very wide but closely related aregiant cell arteritis (with similar histologic features)and sarcoidosis.6Generally, management involves the use of cor-ticosteroids. However, some studies have suggestedIL-6 receptor inhibitors, B-cell depletion, cytotoxicdrugs (especially for steroid resistant or relapsingcases) and anti-tumor necrosis factor agents.10 Openand endovascular graft and bypass surgery haveproven a long-term beneﬁcial eﬀect especiallyFigure 4. Doppler pulsed wave image showing decreased flow velocities in left & right ophthalmic arteries (left panel) andvertebral artery (right panel).Figure 3. Echographic image showing severe occlusion of the left common carotid artery (arrow).Aminde et al. 3 at Glasgow University Library on September 19, 2016shr.sagepub.comDownloaded from in cases with severe stenosis; however, appropriateand timely pharmacologic therapy may limit vascularinterventions. The follow-up of patients shouldinclude control of cardiovascular risk factors espe-cially hypertension, dyslipidemias and also anti-plate-lets aggregants for stroke prevention. Diagnosis ofTA in primary care facilities especially in resource-limited settings like ours is usually very challengingwith late presentation to hospital, absence of state-of-the-art diagnostic imaging tools; with patients at riskof developing advanced vascular lesions and exposingthem to fatal complications.TA is rare among the Africans. In resource-limitedsettings, thorough clinical and doppler echocardio-graphic assessment is important in the diagnosis ofTA with relatively good response to steroids.DeclarationsCompeting interests: None declaredFunding: None declaredGuarantor: LNAEthical approval: Written informed consent for publication wasobtained from the patient.Contributorship: LNA contributed to the initial management ofpatient, conception of case report, data collection and assisted inechocardiography, literature review and writing of ﬁrst draft.JJNN contributed to literature review and critical revision ofmanuscript. NT contributed to literature review and reviewof manuscript. TN contributed to literature review and revisionof manuscript. AD contributed to management of the patient,did the echocardiography and contributed to data collection, crit-ical revision of the manuscript. All authors read and approved theﬁnal manuscript.Figure 5. Doppler image showing significant reduction in flow velocities in left radial (left panel) and ulnar (right panel) arteries.Table 1. American College of Rheumatology (ACR) 1990 criteria for diagnosis of TA.7i. Age at onset less than or equal to 40 yearsii. Claudication of extremities – development and worsening of fatigue and discomfort in muscles of 1 or more extremitywhile in useiii. Decreased brachial artery pulseiv. Systolic blood pressure difference greater than 10 mmHg between armsv. Bruit over subclavian arteries or abdominal aortavi. Arteriographic abnormality: narrowing or occlusion of the entire aorta, its primary branches or large arteries in theproximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia or similar causes; changesusually focal or segmental4 Journal of the Royal Society of Medicine Open 7(8) at Glasgow University Library on September 19, 2016shr.sagepub.comDownloaded from Acknowledgements: NoneProvenance: Not commissioned; peer-reviewed by Zehra ErenReferences1. Johnston SL, Lock RJ and Gompels MM. Takayasuarteritis: a review. J Clin Pathol 2002; 55: 481–486.2. Katsuyama T, Sada K-E and Makino H. Current con-cept and epidemiology of systemic vasculitides. AllergolInt Off J Jpn Soc Allergol 2014; 63: 505–513.3. Yadav P, Prakash P, Srivastava D, Sharma SC andMathews SE. Takayasu’s arterioitis – an unusual pres-entation. J Indian Acad Clin Med 2007; 8: 185–187.4. Kasashima S, Zen Y, Kawashima A, Endo M,Matsumoto Y, Kasashima F, et al. A clinicopathologicstudy of immunoglobulin G4-related sclerosing diseaseof the thoracic aorta. J Vasc Surg 2010; 52: 1587–1595.5. JCS Joint Working Group. Guideline for managementof vasculitis syndrome (JCS 2008). Japanese CirculationSociety. Circ J 2011; 75: 474–503.6. Stone JR, Bruneval P, Angelini A, Bartoloni G, BassoC, Batoroeva L, et al. Consensus statement on surgicalpathology of the aorta from the Society forCardiovascular Pathology and the Association forEuropean Cardiovascular Pathology: I. Inflammatorydiseases. Cardiovasc Pathol 2015; 24: 267–278.7. Arend WP, Michel BA, Bloch DA, Hunder GG,Calabrese LH, Edworthy SM, et al. The AmericanCollege of Rheumatology 1990 criteria for the classifi-cation of Takayasu arteritis. Arthritis Rheum 1990; 33:1129–1134.8. Chung JW, Kim H-C, Choi YH, Kim SJ, Lee W andPark JH. Patterns of aortic involvement in Takayasuarteritis and its clinical implications: evaluation withspiral computed tomography angiography. J VascSurg 2007; 45: 906–914.9. Schmidt WA, Nerenheim A, Seipelt E, Poehls C andGromnica-Ihle E. Diagnosis of early Takayasu arteritiswith sonography. Rheumatol Oxf Engl 2002; 41:496–502.10. Unizony S, Stone JH and Stone JR. New treatmentstrategies in large-vessel vasculitis. Curr OpinRheumatol 2013; 25: 3–9.Aminde et al. 5 at Glasgow University Library on September 19, 2016shr.sagepub.comDownloaded from 

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Takayasu's arteritis in an adult female from Cameroon: diagnosis via Doppler echocardiography

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