RT Journal Article SR Electronic T1 Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis JF RMD Open JO RMD Open FD EULAR SP e001020 DO 10.1136/rmdopen-2019-001020 VO 5 IS 2 A1 Ana F Águeda A1 Sara Monti A1 Raashid Ahmed Luqmani A1 Frank Buttgereit A1 Maria Cid A1 Bhaskar Dasgupta A1 Christian Dejaco A1 Alfred Mahr A1 Cristina Ponte A1 Carlo Salvarani A1 Wolfgang Schmidt A1 Bernhard Hellmich YR 2019 UL http://rmdopen.bmj.com/content/5/2/e001020.abstract AB Objective To collect available evidence on management of large vessel vasculitis to inform the 2018 update of the EULAR management recommendations.Methods Two independent systematic literature reviews were performed, one on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO (population, intervention, comparator and outcome) strategy, Medline, Embase and Cochrane databases were accessed. Eligible papers were reviewed and results condensed into a summary of findings table. This paper reports the main results for Takayasu arteritis (TAK).Results A total of 287 articles were selected. Relevant heterogeneity precluded meta-analysis. Males appear to have more complications than females. The presence of major complications, older age, a progressive disease course and a weaker inflammatory response are associated with a more unfavourable prognosis. Evidence for details on the best disease monitoring scheme was not found. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids are widely accepted as first-line treatment. Conventional immunosuppressive drugs and tumour necrosis factor inhibitors were beneficial in case series and uncontrolled studies. Tocilizumab failed the primary endpoint (time to relapse) in a randomised controlled clinical trial; however, results still favoured tocilizumab over placebo. Vascular procedures may be required, and outcome is better when performed during inactive disease.Conclusions Evidence to guide monitoring and treatment of patients with TAK is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.