British Heart3Journal, I970, 32, I8I.

Non-specific arteritis of the aorta and itsmain branches

P. N. Thenabadu, K. Rajasuriya, and H. R. WickremasingheFromn the University Medical Unit and Department of Pathology,General Hospital, Colombo, Ceylon

Four cases of non-specific arteritis involving the aorta and its main branches are described. Three ofthe cases were hypertensive and one of these had evidence of aortic incompetence. Cases I, 2, and3 had involvement of the aortic arch vessels and the descending aorta, whereas Case 4 presentedas a coarctation of the abdominal aorta. There was a significant association with systemicdisturbance such as polyarthritis, fever, weight loss, raised erythrocyte sedimentation rate, andhyperglobulinaemia. A detailed necropsy in Case 2 showed two large dissecting aneurysms.The nomenclature, the diagnostic criteria, and a probable pathogenesis of the disease are discussedwith reference to the relevant published material.

This paper is concerned with a disease mostlyoccurring in young women, involving theaorta and its main branches and having a well-defined natural history and pathological fea-tures. In I908 Takayasu described peculiarocular changes in a young woman under hiscare. It was Onishi (I908) who, however,showed that a patient with similar ocular chan-ges had in addition absent radial pulses.Though no pathological studies were done,because of the occurrence of the disease inyoung Japanese women it is assumed that thecases described by Takayasu and Onishi wereprobably the first published ones of non-specific arteritis.The disease has been the subject of a large

number of case reports and reviews under avariety of names such as 'reversed coarcta-tion' (Giffin, I939), 'the aortic arch syn-drome' (Fr0vig, I946), 'pulseless disease'(Shimizu and Sano, I95I), 'young femalearteritis' (Ross and McKusick, I953), and'obliterative brachio-cephalic arteritis' (Gib-bons and King, 1957). The fact that the in-volvement of the aortic arch is not a hallmarkof the disease was soon realized when caseswere described with involvement of any partof the aorta and any of its main branches.Cases of aortitis affecting the descendingaorta, described as 'elongate coarctation'(Milloy and Fell, I959) and 'middle aorticsyndrome' (Sen et al., i963), which werethought to be distinct diseases, are probablya manifestation of the same disease entity. In

Received 9 April 1969.5

the search for a suitable term, 'pulselessdisease' has too broad a connotation. Simi-larly, descriptive titles depending on the siteof the aorta or vessels affected are of limiteduse. In the absence of any definite knowledgeon the aetiology, the eponymous term 'Taka-yasu's disease' or a descriptive title of thepathology such as 'idiopathic or non-specificarteritis' should be used.The purpose of this paper is to report 4

cases of Takayasu's arteritis admitted to theUniversity Medical Unit, General Hospital,Colombo. These illustrate the diffuse natureof the aortic involvement and serve to empha-size the systemic nature of the disease and itsclose resemblance to the connective tissue dis-orders. In addition, the presence of dissectinganeurysms in the affected vessels in one of ourcases is a feature not hitherto recorded.

Case I An unmarried 28-year-old woman wasadmitted with a history of effort dyspnoea of fiveyears' duration. Her illness started in I963 with afebrile episode lasting two weeks, followed a weeklater by swelling of the feet and dyspnoea. Shewas treated at hospital and left free of oedema.Since then she complained of loss of weight andof irregular attacks of fever lasting two to threeweeks each. On her next admission to the unitthree years later she was febrile, and had a diminu-tion in the right radial, brachial, and subclavianpulsations. The blood pressures were I6o/ioo mm.Hg in the right arm and 2I0/II5 mm. Hg in theleft. There was cardiomegaly. Erythrocyte sedi-mentation rate was 6o mm. in i hour. Blood cul-

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I82 Thenabadu, Rajasuryia, and Wickremasinghe

tures were repeatedly negative and muscle biopsyshowed no evidence of polyarteritis nodosa.A diagnosis of Takayasu's arteritis was made

and she was treated with guanethidine andprednisolone.On her next admission on 13 May I968, she

was anaemic. The pulsation in her right radial,

FIG. i Arch angiogram in Case I showing a

slightly dilated aorta with obstruction of theright subclavian artery, and narrowing of bothcommon carotid arteries for a variable distancefrom their origin.

FIG. 2 The descending aorta in Case i, show-ing a narrowed and irregular lumen.

TABLE Results of in-vestigations

Investigation Case z Case 2 Case 3 Case 4

Erythrocyte sedimentation rate 55 45 56 25(Ist hour in mm.)

Haemoglobin (g./ioo ml.) 6.4 6z2 6-o 8-6Mantoux Negative Positive Positive Not doneSerological test for syphilis (VDRL) Negative Negative Not done Not doneSerum proteins (total g./xoo ml.) 7T4 7-7 7-2 73Albumin 3-7 3-8 3-7 4.6Globulin 3-7 3.9 3-5 2.7

Serum cholesterol (mg /ioo ml.) I50 Not done Not done Not doneBlood urea (mg./ioo ml.) 2 i6 22 20Blood culture Negative Negative Not done Not doneLatex flocculation test Negative Negative Not done NegativeBlood for lupus erythematosus cells Negative Negative Not done NegativeAntinuclear factor (fluorescent anti- Negative Negative Not done Not donebody technique)

brachial, and subclavian arteries was diminishedcompared to the left. Both carotid pulsations werefelt equally but the wall of the right felt irregularand beaded. The arterial pulsations in both lowerlimbs were weak. The blood pressure in the rightarm was I40/oI0, in the left arm 200/II0, and inthe thighs 170/120 mm. Hg. A systolic bruit washeard at the base of the neck on both sides and inthe epigastrium. The apex beat was heaving andfelt in the anterior axillary line in the seventhintercostal space. The heart sounds were normaland there was a soft ejection systolic murmur atthe base and apex. The right optic fundus wasanaemic compared to the left and digital ophthal-modynamometry revealed a low diastolic pressurein the right fundus as compared to the left.

Pathological investigations of the four cases aresummarized in the Table. In Case I the chest filmshowed generalized cardiac enlargement andnotching of the lower borders of the right thirdand fourth ribs. The electrocardiogram showedevidence of left ventricular hypertrophy and in-complete right bundle-branch block. An intra-venous pyelogram showed that both kidneysexcreted the contrast medium, but the concentra-tion of the dye on the left was less than on theright side. Arch angiography and aortography(Fig. I) showed a dilated aortic arch. The rightsubclavian was obstructed distal to the origin ofthe right vertebral artery. The right and leftcommon carotids were narrowed for a variabledistance from their origin. The left subclavianwas normal and the descending aorta showed anarrowed and irregular lumen (Fig. 2).

Case 2 A woman of 23 years was admitted tothis unit on 3 October I968, with a history ofthree months' effort dyspnoea and swelling offeet. At the age of I2 she had had arthritis of herleft knee lasting 2 weeks. This was not precededby a sore throat. Since then she had had attacksof pain and stiffness of her finger joints for thenext 2 years. She was treated for tuberculouscervical lymphadenitis (diagnosed by lymph nodebiopsy, two years ago) with INAH and PAS forabout one year. Her menstrual history was irregu-lar. She had been married for six years and hadno children.

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Non-specific arteritis of the aorta and its main branches 183

On examination she was a thin frail anaemicwoman with pitting oedema up to the thighs. Shehad lymphadenopathy of the cervical, axillary,and inguinal glands, and finger-clubbing on theright side. The right radial, brachial, and sub-clavian pulsation were diminished as compared tothe left. The abdominal aortic pulsations werenot felt. The femoral pulsations were diminishedand delayed. The popliteal, dorsalis pedis, andposterior tibial pulsations were not felt. Theblood pressure in the right arm was 80/50, in theleft arm I40/50, and in the thighs IIO/60 mm. Hg.She was in congestive heart failure. There wascardiomegaly with biventricular enlargement.There was a proto-diastolic gallop rhythm heardin the left third space, a blowing early diastolicmurmur in the aortic area and at the left sternaledge, with a soft ejection systolic murmur in thepulmonary area. Systolic bruits were also audibleover the subclavian arteries in the supraclavicularfossae and to the left of the spine at about Dio.Her spleen was palpable one finger below the leftcostal margin.

InvestigationsChest x-ray showed generalized cardiomegaly withclear lung fields. Electrocardiogram showed leftaxis deviation, left ventricular hypertrophy, andT inversion in leads VI, V2, and V4. Aortographyand cine-angiography showed a dilated ascendingaorta, aortic incompetence with normal valvularmechanism, partial obstruction of right subclavianat its origin, and narrowing of right common caro-tid (Fig. 3). There was also narrowing of thedescending abdominal aorta above the level of therenal arteries.

FIG. 3 Arch angiogram in Case 2, showing adilated aorta, obstruction of the right sub-clavian artery at its origin, and narrowing ofthe right common carotid artery.

FIG. 4 Case 2. Irregular dilatation of theaorta with dissecting aneurysms in the des-cending aorta and right subclavian arteries.

FIG. 5 Case 2. The cut section of the dissectinganeurysm on the descending aorta showingblood clot and the laminated appearance ofthe fibrous capsule.

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x84 Thenabadu, Rajasuriya, ar;d Wickremasinghe

She was again readmitted in advanced congestiveheart failure and died on 22 January I969.

NecropsyThe heart was enlarged. The left ventricular wallwas hypertrophied (I-5 cm.). The right ventricu-lar wall, the atria, the atrioventricular valves, andthe endocardium showed no abnormality.The entire thoracic aorta starting from the

valve ring was irregularly dilated (Fig. 4). A firmswelling about 3 cm. in diameter was observed atthe origin of the right subclavian. Sections of thisshowed laminated firm white tissue with soft yel-low and brown material in the centre. A largeswelling, ovoid, about 7 cm. in length and about4 cm. in the largest diameter, was present in thethoracic aorta just above the diaphragm. Thisconsisted of laminated white tissue with haema-toma in the lower pole (Fig. 5). Both these massesreduced the lumen of the vessels to a thin slit butneither of them had any communication with thelumen. The intimal surface of the aorta had acobbled appearance due to patchy fibrous orfleshy thickening (Fig. 6). No atheromatous chan-ges of the endothelium were observed. The otherarterial branches arising directly from the aortawere normal, as were the coronary and cerebralarteries.

FIG. 6 Case 2. Cut section of the aorta showingthe irregular cobbled appearance of the intima.There is no communication with the dissectinganeurysm (arrow). The two openings seen arethe ostia of two intercostal arteries.

FIG. 8 Case 2. Section from descending aorta,showing collection of lymphocytes andhistiocytes in the media. (Haematoxylin andeosin. x 450.)

FIG. 7 Case 2. Section from descending aortashowing medial fibrosis with fragmentation ofelastic. Intimal and adventitial fibrosis is alsoprominent. (Elastic van Gieson. x 25.)

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Non-specific arteritis of the aorta and its main branches I85

Histology Aorta: the intima was conspicuouslythickened in some areas by fibrosis. The mediawas irregularly thinned and showed destructionor absence of elastic and muscle fibres with re-placement fibrosis (Fig. 7). In some areas numer-ous vascular channels with perivascular chronicinflammatory cells were seen in the atrophicmedia (Fig. 8). The adventitia showed fibrousthickening and collection of chronic inflammatorycells around the vasa vasorum. There was noendarteritis of these vasa. No evidence of athero-ma, cystic medial necrosis, or fibrinoid necrosiswas seen in any of the sections examined.The mass in the thoracic aorta was an organiz-

ing haematoma dissecting its wall. It consisted ofa central zone of fibrin and red cells, a zone ofgranulation tissue with many chronic inflamma-tory cells outside this, and a peripheral zone offibrous tissue. The histology of the mass in theright subclavian artery was similar, except for thepresence of large collections of lipid-containingphagocytes in the wall. The other main branchesof the aorta, the pulmonary artery and vein, andthe coronary artery were histologically normal.

Heart: the left ventricle showed hypertrophicmuscle fibres. The endocardium, pericardium, andinterfascicular connective tissue were normal.

Case 3 A woman aged 29 years was admittedto this unit on 23 September I968, with a historyof intermittent claudication of the lower limbs oftwo weeks' duration. Her claudication distancewas about I0 yards and the pain was felt mainlyin the calf muscle. One month previously she hadfever with chills, and dysuria. In spite of treat-ment by a general practitioner she had continuedto get the fever with chills about twice a week.She also gave a history of headache and pain inthe small joints of her hands with stiffness, pain,and swelling of the left knee two years before.This subsided with Auyrvedic treatment.' Shewas married, and had 6 children of whom 4 wereliving.On examination she was a pale thin woman who

had no webbing of the neck or increase in thecarrying angle of the elbows. Both femoral pulseswere weak and delayed. The popliteal and dor-salis pedis pulsations were not felt. The bloodpressure in the right arm was i80/I25, in the leftarm I30/I00, and in the thighs I30/110 mm. Hg.The heaving apex beat was in the fifth intercostalspace I-25 cm. outside the midclavicular line. Asoft ejection systolic murmur was followed by aloud aortic second sound. Ophthalmoscopyshowed grade i hypertensive changes. X-rays ofthe chest and abdomen were normal. Electro-cardiogram showed left ventricular hypertrophyand strain. The patient refused aortography.

Case 4 A girl aged 13 years was admitted tothis unit from another hospital on i8 May I967,with a history of joint swelling of one-and-a-halfyears' duration, and headache and effort dyspnoeafor the previous one year.1 The indigenous system of medicine in Ceylon.

On examination she was anaemic and febrileand showed generalized lymphadenopathy. Therewas no webbing of the neck or increased angle ofthe elbows. The pulsations of the peripheralarteries were normal and there was no femoraldelay. The blood pressure in both arms was 220/I6o mm. Hg, and in the thighs 200/150 mm. Hg.A systolic murmur was heard in the abdomen,and to the right of the first lumbar vertebra pos-teriorly. The heart was not clinically enlarged.The left elbow joint was ankylosed at 450 andinflamed. There was inflammatory swelling of theright ankle joint and spindling of the interphal-angeal joints. The fundi showed grade i retino-pathy.The teleradiogram showed a slightly enlarged

heart and widening of the ascending aorta. Elec-trocardiography showed left ventricular hyper-trophy. A transfemoral aortogram showed coarc-tation of the aorta at the level of the renal arteries(Fig. 9).

FIG 9 Aortogram in Case 4 showing narrow-ing of the descending aorta and non-opacifica-tion of the right renal artery.

At operation under moderate hypothermiathere was a coarctation of the abdominal aorta atthe level of the renal arteries, with adequate pul-sations and flow distal to the coarctation. Theright renal artery was very small with no pulsa-tion. Left renal artery pulsation was good. A right-sided nephrectomy was done. The blood pressuredropped to I6o/Ioo mm. Hg soon after nephrec-tomy but later rose to I60/I20 mm. Hg.

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x86 Thenabadu, Rajasuriya, and Wickremasinghe

DiscussionThese four cases illustrate the protean clinicalmanifestations of Takayasu's disease. Thecharacteristic features which help in the diag-nosis of this condition are first, a strong pre-dilection for affecting young women or girls.It is more prevalent in Asian and Africancountries than in the West (McKusick, I962).

Secondly, apart from the arteritis, there isevidence of a systemic disturbance. The factthat this is essentially a generalized diseasewith a predilection for the aorta and its mainbranches was emphasized by Strachan (I964)in a review ofthe natural history ofthe disease.There is often irregular fever, fatigue, andweight loss, as seen in our Cases i, 2, and 3.A history of arthritis was elicited in Cases 2,3, and 4. This was usually of a rheumatoidtype but in all cases the flocculation tests werenegative. Splenomegaly (Miller, Thomas, andMedd, I962), and generalized lymphadeno-pathy have been rarely reported in this disease,and these were seen in Cases 2 and 4, respec-tively. All four patients in this series were

anaemic and had a raised erythrocyte sedi-mentation rate. A hyperglobulinaemia was

seen in Cases I, 2, and 3, with a predominanceof ,B- and y-globulins in Case 2 (Table).

Thirdly, there is involvement of the aortaand its main branches. This affection is vari-able and may involve any part of the aortaleading to obstruction by inflammatory nar-

rowing or thrombosis, or it may lead to dilata-tion and aneurysm formation. Aortitis isdiffuse, though in the early stage, when one

vessel or one segment of the aorta is affected, itmay be mistaken for a congenital anomaly.Cases 3 and 4, for example, presented a coarc-

tation of the abdominal aorta and may havebeen mistaken for a congenital anomaly; thisis, however, rare in female patients unlessassociated with Turner's syndrome. More-over, Case 3 has involvement of the left sub-clavian artery and Case 4 of the right renalartery, and in both cases there was definiteevidence of a systemic disturbance. Thearterial involvement must also be distin-guished from conditions such as atherosclero-sis, syphilis, polyarteritis nodosa, giant cellarteritis, and dissecting aneurysm of the aorta.In the early and difficult cases, follow-up, or

histological study if possible, usually dis-closes the true nature of the condition. Thevessels affected may be suspected from theischaemic symptoms, from reduced peripheralarterial pulsations, and from the differentblood pressures found in the affected limbs.But the true extent of the involvement can

only be determined by angiographic studies.

The fourth diagnostic criterion would behistological evidence obtained either by biopsyor at necropsy, but we must stress that a diag-nosis cannot be made on such evidence alone.Biopsy is usually not possible owing to thesegmental nature of the arterial involvementand the difficulty in obtaining suitable speci-mens. As pointed out by Judge et al. (I962),confusion can be avoided in the interpretationof the histopathology if one remembers thevariability in the stage of the disease. Judgeet al. (I962) conclude that the pathologicalprocess begins as a periarteritis and progressesto a panarteritis. Varying degrees of inflamma-tion and repair would be seen in the variouscoats eventually leading to sclerosis. Ourpathological findings in Case 2 are essentiallyin agreement with these observations, but wehave not found evidence that there is a centri-petal sequence of involvement. The extent ofinvolvement of the three coats does not sug-gest such a sequence. We observed, for in-stance, gross intimal fibrosis and thickening,with minimal involvement of the media andadventitia. Nor are our observations consistentwith the views of Judge et al. (I962) and Mar-quis et al. (I968) that the fibrosis and intimalthickening are secondary to the destructivechanges in the media.

Cardiomegaly was seen in all our cases.The heart is involved in this disease eitherthrough hypertension, as in Cases I, 3, and 4,through aortic incompetence, as in Case 2,through obstruction of the coronary arteriesat their ostia (Barker and Edwards, I955; Rossand McKusick, 1953), or through coronaryarteritis (Danaraj, Wong, and Thomas, I963).Hypertension is a common accompanimentof the disease and is usually due to renalartery narrowing or results from the coarcta-tion of the aorta itself. Cardiac involvementor the development of hypertension are im-portant events in the natural history of thedisease, as their presence worsens the prog-nosis.

Case 2 was of interest because of the pre-sence of two dissecting aneurysms, neither ofwhich were shown on angiography; and thereason for this was apparent at necropsy whereno communication with the parent vessel wasseen. Though an intimal tear is not alwaysshown, if absent the dissecting aneurysm islikely to be produced by destructive lesionsof the medial vasa vasorum, producing anorganized haematoma. The laminated struc-ture of the wall of the dissecting haematomaalso lends support to this view. While fusi-form and saccular dilatations of the affectedvessels have been described in Takayasu's

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Non-specific arteritis of the aorta and its main branches 187

disease, we have not come across a single pub-lished case of dissecting aneurysm in Taka-yasu's disease. In giant cell aortitis, where thehistological appearance of the media can re-semble that in Takayasu's disease, dissectinganeurysms are not uncommon (McMillan,1950; Magarey, 1950; Paulley and Hughes,I960; Harris, I968). The intimal fibrous thick-ening in giant cell aortitis is never so conspicu-ous as in Takayasu's disease, and this differ-ence may be of significance in view of therarity of dissecting aneurysms in Takayasu'sdisease.The frequent association ofthis disease with

tuberculosis has been observed earlier (Senet al., I963; Danaraj et al., I963), but inAsian countries where there is a high incidenceof tuberculosis such an association to be sig-nificant must have statistical support. Case 2in the present series had been treated fortuberculosis of the cervical lymph nodes.Occasional cases with the features of Taka-yasu's disease and a positive lupus erythema-tosus cell test have been observed by Lessofand Glynn (I959), Judge et al. (I962), andMiller et al. (I962). However, these occasionalcases with positive results are of little signifi-cance, as in most connective tissue disordersthe lupus erythematosus cell phenomenon ismore often negative. In fact, the lupus ery-thematosus cell test and the antinuclear factorhave been negative in our cases. The associa-tion of overt rheumatoid arthritis with Taka-yasu's arteritis has been reported by Falicovand Cooney (1964) in a case of juvenile rheu-matoid arthritis, and by Sandring and Welin(I96I) in three cases - two in adults and onewith juvenile rheumatoid arthritis. Case 4can be considered as another instance of theassociation of Takayasu's arteritis with Still'sdisease. However, the association of Taka-yasu's arteritis with transient arthritis andarthralgia (as in Cases 2 and 3) and other'rheumatic' symptoms has been recordedmore frequently (Ask-Upmark, 1954; San-dring and Welin, I96I; Birke, Ejrup, and 01-hagen, I957; Strachan, I964; Schrire andAsherson, I964). Judge et al. (I962) suggestthat this may be a connective tissue disorderwith an auto-immunopathy affecting vascularelastin. However, most pathological studies,including the present one, indicate a morediffuse involvement of all the coats of thearterial wall than would be the case if elastinwere primarily affected. Here we would againemphasize the dissociation of the intimal,medial, and adventitial changes. If an auto-immunopathy or other process primarilyaffecting a specific tissue element is to be in-voked, the basophilic ground substance in

which elastic, muscle, and collagen are allembedded appears more likely to be the targetthan elastin. It is significant that this baso-philic ground substance is normally presentin appreciable amounts in the aorta and thearteries affected in Takayasu's disease.

Our sincere thanks to Mr. A. T. S. Paul, GeneralHospital, Colombo, for operating on Case 4, toDr. M. Weerasena and Dr. M. Jayasinghe for thearteriographic studies, and the Medical Superin-tendent, General Hospital, Colombo, for per-mission to publish.

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side of Japan. Acta Medica Scandinavica, 149, i6i.Barker, N. W., and Edwards, J. E. (I955). Primary

arteritis of the aortic arch. Circulation, II, 486.Birke, G., Ejrup, B., and Olhagen, B. (1957). Pulseless

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Danaraj, T. J., Wong, H. O., and Thomas, M. A,(I963). Primary arteritis of the aorta causing renalartery stenosis and hypertension. British HeartJournal, 25, 153.

Falicov, R. E., and Cooney, D.-F. (i964). Takayasu'sarteritis and rheumatoid arthritis. Archives of Inter-nal Medicine, 114, 594.

Frovig, A. G. (I946). Bilateral obliteration of the com-mon carotid artery. Thromboangiitis obliterans?Acta Psychiatrica et Neurologica Scandinavica,Suppi. 39.

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McKusick, V. A. (i962). A form of vascular diseaserelatively frequent in the Orient. American HeartJournal, 63, 57.

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Magarey, F. R. (1950). Dissecting aneurysm due togiant-cell aortitis. J3ournal of Pathology and Bacteri-ology, 62, 445.

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