Pre-pulseless and Pulseless Takayasus' Arteritis

The Confused Terminology and Changing Concept of Takayasu's Arteritis In 1908 the Japanese opihthalmologist, Takayasu, reported an unusual wreath-like vascular anastomosis surrounding the optic discs of a young female patient who was nearly iblind, biut who showed 'no general physical changes'. He was unalble to explain this abnormality. Laiter a colleague, Onishi, referred to a similar case who had the further 'peculiarity' of pulselessness of the arms. Eventually it was established that these widely separated abnormalities were ibut 'two of many remarkable features which could occur when disease caused severe stenosis or obstruction of 'the arterial trunks arising from fthe aortic arch. The 'full spectrum, whlich is best termed the aortic-arch syndrome (Frovig, 1946), included bizarre features such as premature aging of the face, masseter claudication and ulceration of the palate, nasal septum, ears or nose in addition t'o the commoner consequences of ocular, cerebral and 'arm ischaem'ia sucih 'as iblindness, strokes and arm claudication. Because of the rarity of aitheroma in ithe Orient it became apparent early 'that the aortic-arch syndrome could be due to an iarteritis of obscure aetiology. In the absence, however, of aortography or ispecific serological tests neither early diagnosis nor accurate distinction of this 'arterial disease from the other causes of 'the syndrome such as syp1hilis, polyarteriitis, chronic dissecting aneurysm or 'atheroma was possiible. During the first half of this century these circumstances and the variable presenitations 'of 'the aortic-arch syndrome obscured the early niatural history of this new 'arteritis 'and led to nosoilogical confusion (Table 1). Unfortunately, the (misleading term 'pul;seless dilsease' gradually 'became dominan't, being used by some as a synonym for the syndrome and by others specifically for the arteries first referred to, lalbeit unknowingly, 'by either Takayasu or Savory (1856). The last decade has added to the confusiion since descriptions of Takayasu's arteri'tis distal to the alortic arch have resulted in a further selection 'of itermls. These include central aortitis, middle (aortic syndrome, stenosing aortitis, enilongated coarctation and subisthmic coarctation. Largely because of the term 'pulseless disease' Tlakayasu's ariteritis came to,be regarded essen'tiially as a cause 'of arm ipuliselessness, blindness or ce[robro-vasculiar insufficiency. Recently, however, several papers (Sandring and Welin, 1961; Judge, Currier, Gracie and Figley, 1962; Strachan, 1964; TABLE 1 TERMINOLOGY OF TAKAYASU'S ARTERITIS Takayasu's disease Pulseless disease Takayasu;Onishi disease Branohial arteritis Reader-Harbitz sy.ndrome Arteritis of ao,rta in young women Aortic arch arteriltis Syndrome of occlusion of supra-aortic trunks Young female arteritis Rheumatic brachio-vephalic arteritis Reversed coarctation Martorell's syndrome Takayasu-Martorel.l-Fa'bre syndromo Aoritic arch syndrome Thrombo-arteritis obliterans subclavico-carotica Chronic subolavian-carotid obstructive syndrome