Takayasu arteritis: When rarity maintains the mystery

Takayasu arteritis (TA) is a rare vasculitis involving the large arteries and often recognized with significant delay. The etiology of TA is unknown. Young women are more frequently affected, with significant mortality and morbidity. The clinical picture is heterogeneous and mainly determined by arterial involvement, in terms of arterial steno-occlusions or dilatations. Unfortunately, TA is still an orphan disease, and many issues remain open. Multiple reasons limit the studies in this field: the rarity of TA, the difficulty in obtaining tissue specimens, the lack of informative animal models, the need of accurate outcome measures and of adequate techniques to evaluate TA proteiform activity. Here we review principles of TA management and discuss some major open issues, underlining the need to overcome the simplistic view of “TA as a purely inflammatory condition”, to recognise the important role of arterial remodelling as a consequence of arterial inflammation and injury.