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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.

Original publication




Journal article


RMD open

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Rheumatology, Centro Hospitalar do Baixo Vouga EPE, Aveiro, Portugal.


Humans, Disease Susceptibility, Diagnosis, Differential, Prognosis, Treatment Outcome, Combined Modality Therapy, Severity of Illness Index, Comorbidity, Patient-Centered Care, Disease Management, Takayasu Arteritis, Giant Cell Arteritis, Symptom Assessment, Biomarkers