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.
- Giant Cell Arteritis
- Systemic vasculitis
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Contributors AFÁ and SM conducted the SLRs. AFÁ drafted the first version of the current manuscript and subsequent revisions. All authors reviewed and approved the final version of the manuscript.
Funding This project was funded by EULAR.
Competing interests SM received speaker fees and consultancies from Roche and Chugai. FB received grants from Horizon and Mundipharma, and speaker fees and/or consultancies from Horizon, Mundipharma, Roche and Sanofi. MC received lecturing fees from Boehringer Ingelheim and Vifor, consulting fees from Roche, Janssen, AbbVie and GSK, and research agreement from Kiniksa. CD received a grant from Celgene, and speaker fees and/or consultancies from AbbVie, BMS, Lilly, MSD, Pfizer, Novartis, UCB, Roche and Sanofi. WS received a grant from Roche, and speaker fees and consultancies from Chugai, GSK, Novartis, Roche and Sanofi. BH received speaker fees and/or consultancies from AbbVie, Boehringer, Chugai, Celgene, MSD, Pfizer, Novartis and Roche. All other authors have no competing interests.
Patient consent for publication Not required.
Ethics approval Since this project was an SLR and did not use individual patient data, ethical approval was not deemed necessary.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.
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