title: Updates in Pathophysiology, Diagnosis and Management of Takayasu Arteritis.
abstract: BACKGROUND
  Takayasu arteritis (TA) is a rare, systemic, inflammatory vasculitis of granulomatous nature, and still of unknown etiology. It mainly involves the aorta and its major branches and is more commonly seen in women of childbearing age and Asians. TA leads to stenosis, occlusion, or aneurysmal degeneration of large arteries, and its pathogenesis seems to be mainly due to an abnormal cell-mediated immunity, although other molecular and genetic abnormalities may contribute. The diagnosis and treatments lie on clinical and arteriographic findings. Because of its fluctuating course, both clinical scores and biomarkers are currently evaluated. The aim of this review is to report a comprehensive and methodologically robust state of the art about Takayasu arteritis, including the latest data and evidences in the definition, epidemiology, pathogenesis and etiology, clinical manifestations and classification, diagnosis, assessment of disease activity and progression, biomarkers, and treatment.
  
  
  METHODS
  We searched all publications addressing definition, epidemiology, pathogenesis, etiology, classification, diagnosis, biomarkers, and treatment of TA. Randomized trials, cohort studies, and reviews were contemplated to give a breadth of clinical data. PubMed and Scopus were searched from August 2010 to November 2015.
  
  
  RESULTS
  Of the 3,056 records found, 267 matched our inclusion criteria. After reading the full-text articles, we decided to exclude 169 articles because of the following reasons: (1) no innovative or important content; (2) no multivariable analysis; (3) insufficient data; (4) no clear potential biases or strategies to solve them; (5) no clear end-points; and (6) inconsistent or arbitrary conclusions. The final set included 98 articles.
  
  
  CONCLUSIONS
  This review presents the last updates in all fields of Takayasu arteritis. Still today, large areas of TA pathogenesis and disease-activity assessment need to be further investigated to better treat patients with TA.
authors.affiliation: Interuniversity Center of Phlebolymphology (CIFL), International Research and Educational Program in Clinical and Experimental Biotechnology, Headquarters: University Magna Graecia of Catanzaro, Catanzaro, Italy; Department of Medical and Surgical Sciences, University of Catanzaro, Catanzaro, Italy. Electronic address: rserra@unicz.it.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Serra
authors.affiliation: Interuniversity Center of Phlebolymphology (CIFL), International Research and Educational Program in Clinical and Experimental Biotechnology, Headquarters: University Magna Graecia of Catanzaro, Catanzaro, Italy; Department of Medical and Surgical Sciences, University of Catanzaro, Catanzaro, Italy.
authors.forename: Lucia
authors.initials: L
authors.lastname: Butrico
authors.affiliation: School of Medicine, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Fugetto
authors.affiliation: School of Medicine, Kazan State Medical University, Tatarstan Republic, Russian Federation.
authors.forename: Mariia Dmitrievna
authors.initials: MD
authors.lastname: Chibireva
authors.affiliation: School of Medicine, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Malva
authors.affiliation: Cardiovascular and Thoracic Department, G. Martino University Hospital, University of Messina, Messina, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: De Caridi
authors.affiliation: Cardiovascular and Thoracic Department, G. Martino University Hospital, University of Messina, Messina, Italy.
authors.forename: Mafalda
authors.initials: M
authors.lastname: Massara
authors.affiliation: Department of Medical and Surgical Sciences, University of Catanzaro, Catanzaro, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Barbetta
authors.affiliation: Department of Surgery, Annunziata Hospital of Cosenza, Cosenza, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Cannistrà
authors.affiliation: Interuniversity Center of Phlebolymphology (CIFL), International Research and Educational Program in Clinical and Experimental Biotechnology, Headquarters: University Magna Graecia of Catanzaro, Catanzaro, Italy; Department of Medical and Surgical Sciences, University of Catanzaro, Catanzaro, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: de Franciscis
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D011237:Predictive Value of Tests; D012307:Risk Factors; D013625:Takayasu Arteritis / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome