OBJECTIVES: To summarize some of the literature about Takayasu arterit
is, 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;sympt
oms of this disease are also available. Because : of nonspecific patho
logy, a rather nonspecific initial clinical presentation and an obliga
tory criterion of age (40 years or younger) it is possible that the di
sease may be underdiagnosed in Europe and North America. The cause of
this disease _ remains obscure. Various infections have been blamed bu
t genetic and immunological disturbances seem to play a major role in,
bringing Takayasu arteritis into the list of autoimmune diseases. INT
ERVENTIONS: Takayasu arteritis responds well to glucocorticoids/cyclop
hosphamide in the acute (prepulseless) phase. In the chronic fibrotic
phase, treatmeat of hypertension and various angioplastic and surgical
interventions are CONCLUSIONS: Adoption of improved diagnostic criter
ia may change the prevailing view that Takayasu arteritis is an Asian
disease. Understanding of the pathogenesis at cellular and molecular l
evels is needed. Creation of an animal model would be a desirable tool
in that direction.