Aortitis syndrome named in Japan is widely known as Takayasu's arteritis internationally. Based on the experiences accumulated since the report of eyeground changes by Takayasu, it has become clear that the clinical manifestations of the disease are quite variable, including pulseless disease, atypical coarctation of the aorta, renovascular hypertension, aneurysms, aortic regurgitation and coronary artery disease. Pulmonary artery involvement is not infrequently present. For an exact diagnosis, it must be kept in mind that two or more of these manifestations are combined in most of the patients. The data of several epidemiological studies are presented and some of the recent literature reviewed.