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Original article
Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis
  1. Ana F Águeda1,
  2. Sara Monti2,3,
  3. Raashid Ahmed Luqmani4,
  4. Frank Buttgereit5,
  5. Maria Cid6,
  6. Bhaskar Dasgupta7,
  7. Christian Dejaco8,9,
  8. Alfred Mahr10,
  9. Cristina Ponte11,12,
  10. Carlo Salvarani13,
  11. Wolfgang Schmidt14 and
  12. Bernhard Hellmich15
  1. 1 Rheumatology, Centro Hospitalar do Baixo Vouga EPE, Aveiro, Portugal
  2. 2 Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy
  3. 3 University of Pavia, PhD in Experimental Medicine, Pavia, Italy
  4. 4 Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMS), University of Oxford, Oxford, UK
  5. 5 Rheumatology and Clinical Immunology, Charité University Medicine Berlin (CCM), Berlin, Germany
  6. 6 Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  7. 7 Rheumatology, Southend University Hospital, Westcliff-on-Sea, UK
  8. 8 Rheumatology, Department of Rheumatology, South Tyrol Health Trust, Hospital of Bruneck, Bruneck, Italy
  9. 9 Rheumatology, Medical University Graz, Graz, Austria
  10. 10 Internal Medicine, Hospital Saint-Louis, University Paris Diderot, Paris, France
  11. 11 Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
  12. 12 Rheumatology, Centro Hospitalar de Lisboa Norte, EPE, Hospital de Santa Maria, Lisboa, Portugal
  13. 13 Rheumatology, Università di Modena e Reggio Emilia and Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
  14. 14 Medical Centre for Rheumatology, Klinik für Innere Medizin, Rheumatologie und Klinische Immunologie Berlin-Buch, Immanuel Krankenhaus, Berlin, Germany
  15. 15 Klinik für Innere Medizin, Rheumatologie und Immunologie, Vaskulitis-Zentrum Süd, Medius Kliniken, – Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim-unter-Teck, Germany
  1. Correspondence to Dr Ana F Águeda; filipaa729{at}


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
  • Treatment
  • 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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