Objectives: To summarize some of the literature about Takayasu arteritis, a nonspecific chronic inflammatory disease involving the aorta and its main branches, and to analyze the criteria for its diagnosis. The pulmonary artery and its branches, as well as the coronary arteries, may also be involved.
Data analysis: Although Takayasu arteritis has been commonly reported in Asian countries such as Japan, India, China and Korea, case reports from other parts of the world describing symptoms of this disease are also available. Because of nonspecific pathology, a rather nonspecific initial clinical presentation and an obligatory criterion of age (40 years or younger) it is possible that the disease may be underdiagnosed in Europe and North America. The cause of this disease remains obscure. Various infections have been blamed but genetic and immunological disturbances seem to play a major role in bringing Takayasu arteritis into the list of autoimmune disease.
Interventions: Takayasu arteritis responds well to glucocorticoids/cyclophosphamide in the acute (prepulseless) phase. In the chronic fibrotic phase, treatment of hypertension and various angioplastic and surgical interventions are required.
Conclusions: Adoption of improved diagnostic criteria may change the prevailing view that Takayasu arteritis is an Asian disease. Understanding of the pathogenesis at cellular and molecular levels is needed. Creation of an animal model would be a desirable tool in that direction.