The role of radiology in the diagnosis of Takayasu's arteriopathy (“pulseless disease”)

We describe three cases of Takayasu's arteriopathy investigated by aortography and peripheral arteriography. Although Takayasu's arteriopathy shows a predilection for the aorta and its branches it is essentially a generalised arterial disease of protean symptomatology and prolonged course. The early phase of its natural history is characterised by systemic symptoms similar to those of systemic lupus erythematosus. As there is frequently no abnormality of the arterial pulses at this time there is little to suggest an arterial disease and the diagnosis is readily overlooked by the clinician. Appreciation of these points by the radiologist is of fundamental importance since careful examination of the thoracic aorta in appropriate cases will often allow the diagnosis to be suggested at an early stage. Segmental dilatation of the descending thoracic aorta is an early hallmark of the disease which may be seen on plain radiographs provided that the exposure is such that the descending aorta can be seen within the heart shadow. Dilatation of the ascending thoracic aorta is equally suggestive, although in its early stages this is more difficult to assess on the plain radiograph. Contrast radiography will demonstrate these features clearly in doubtful cases and is always necessary for confirmation of the diagnosis. During the late occlusive phase, arterial stenosis may cause the stroke syndrome, superior mesenteric artery stenosis, renal artery stenosis or intermittent claudication. The radiological features of a thoracic aortitis may again be an invaluable indication of the true underlying pathology. Rib notching and clacification of the descending thoracic or abdominal aorta may also be seen at this time. Contrast radiography of the entire aorta and its major branches is necessary to define the exact extent and character of the arterial abnormalities and so permit a decision regarding the relative roles of corticosteroid drugs, anticoagulant therapy and surgery in the treatment of the individual case.