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Takayasu Arteritis: When Rarity Maintains the Mystery

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Systemic Vasculitides: Current Status and Perspectives

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Abstract

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.

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Authors and Affiliations

  1. Università Vita-Salute San Raffaele, Via Olgettina 60, 20132, Milan, Italy

    Enrico Tombetti, Angelo A. Manfredi & Maria Grazia Sabbadini

  2. San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy

    Enrico Tombetti, Elena Baldissera, Angelo A. Manfredi & Maria Grazia Sabbadini

Authors
  1. Enrico Tombetti
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  2. Elena Baldissera
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  3. Angelo A. Manfredi
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  4. Maria Grazia Sabbadini
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Corresponding author

Correspondence to Enrico Tombetti .

Editor information

Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Tombetti, E., Baldissera, E., Manfredi, A.A., Sabbadini, M.G. (2016). Takayasu Arteritis: When Rarity Maintains the Mystery. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_6

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