Summary
The clinical spectrum of diffuse arteritis of the aorta and its main branches has been reviewed in a series of 34 patients.
The inflammatory process leads to intimal proliferation, atheroma and superimposed thrombosis, with the result that the lumina of the aorta or its branches are narrowed or even blocked. Weakening of the vessel wall with aneurysm formation is less common. The lesion is diffuse but patchy, so that several vascular territories are simultaneously affected, but to different degrees. Combinations of stenosis and aneurysm formation are also encountered.
The commonest symptoms are cardiac, mainly secondary to hypertension, but coronary arterial and myocardial factors also play a part. Mitral and aortic incompetence occurs, but has no hæmodynamic significance. Involvement of the main branches of the aorta is the hallmark of the disease, resulting in pulse abnormalities and vascular murmurs. When the upper limb vessels are involved, the anastomotic circulation is usually adequate, but the blood pressure is difficult to record with the sphygmomanometer, so that central aortic pressure tends to be underestimated. Involvement of the lower limbs gives rise to claudication and nutritional disturbances of the affected members. Involvement of the carotid arteries and of the mesenteric and the renal vessels produces symptoms due to ischæmia of the organs supplied by these channels. Renal involvement results in hypertension which aggravates the disease, but other factors contribute to the hypertension.
The angiographic appearances in 27 cases are described. A new entity, the so‐called “coronary steal” syndrome, has been found in two cases. A rich anastomosis between the coronary arteries and the diseased part of the descending thoracic aorta acts as a potential diversion of blood from the heart to the aorta.