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2018 Update of the EULAR recommendations for the management of large vessel vasculitis
  1. Bernhard Hellmich1,
  2. Ana Agueda2,
  3. Sara Monti3,
  4. Frank Buttgereit4,
  5. Hubert de Boysson5,
  6. Elisabeth Brouwer6,
  7. Rebecca Cassie7,
  8. Maria C Cid8,
  9. Bhaskar Dasgupta9,
  10. Christian Dejaco10,11,
  11. Gulen Hatemi12,
  12. Nicole Hollinger13,
  13. Alfred Mahr14,
  14. Susan P Mollan15,16,
  15. Chetan Mukhtyar17,
  16. Cristina Ponte18,19,
  17. Carlo Salvarani20,
  18. Rajappa Sivakumar21,
  19. Xinping Tian22,
  20. Gunnar Tomasson23,
  21. Carl Turesson24,
  22. Wolfgang Schmidt25,
  23. Peter M Villiger26,
  24. Richard Watts27,
  25. Chris Young28,
  26. Raashid Ahmed Luqmani29
  1. 1 Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken, University of Tübingen, Kirchheim-Teck, Germany
  2. 2 Rheumatology Department, Centro Hospitalar do Baixo Vouga E.P.E, Aveiro, Portugal
  3. 3 Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
  4. 4 Department of Rheumatology and Immunology, University Hospital Charité, Berlin, Germany
  5. 5 Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, Basse-Normandie, France
  6. 6 Rheumatology and Clinical Immunology, UMCG, Groningen, The Netherlands
  7. 7 Leicester, UK
  8. 8 Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
  9. 9 Rheumatology, Southend Hospital NHS Trust, Westcliff-on-sea, UK
  10. 10 Rheumatology, Medical University Graz, Graz, Austria
  11. 11 Rheumatology, Hospital of Bruneck, Bruneck, Italy
  12. 12 Division of Rheumatology, Department of Internal Medicine, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey
  13. 13 Department of Internal Medicine, Rheumatology and Immunology, Medus Klinken, Karl-Albrechts-Universität Tübingen, Kirchheim-Teck, Germany
  14. 14 Hospital Saint-Louis, University Paris Diderot, Paris, France
  15. 15 Ophthalmology, University Hospitals Birmingham, Birmingham, UK
  16. 16 Neurometabolism, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
  17. 17 Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
  18. 18 Rheumatology, Hospital de Santa Maria - CHLN, Lisbon Academic Medical Centre, Lisbon, Portugal
  19. 19 Rheumatology Research Unit; Instituto de Medicina Molecular, Instituto de Medicina Molecular, Lisboa, Portugal
  20. 20 Arcispedale S Maria Nuova, Reggio Emilia, Italy
  21. 21 Stroke and Neurocritical Care, GLB Hospitals and Acute Stroke Centers, Chennai, India
  22. 22 Rheumatology, Peking Union Medical College Hospital, Beijing, China
  23. 23 University of Iceland, Reykjavik, Iceland
  24. 24 Department of Rheumatology, Skåne University Hospital, Malmö, Sweden
  25. 25 Medical Centre for Rheumatology Berlin-Buch, Immanuel Krankenhaus Berlin, Berlin, Germany
  26. 26 Rheumatology and Clinical Immunology / Allerg, University Hospital (Inselspital), Bern, Switzerland
  27. 27 Norwich Medical School, Bob Champion Research and Education Building, University of East Anglia, Norwich, UK
  28. 28 Steyning, UK
  29. 29 Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, UK
  1. Correspondence to Prof Dr Bernhard Hellmich, Klinik für Innere Medizin, Rheumatolgie und Immunologie, Medius Kliniken, Eberhard Karls Universität Tübingen, 73230 Kirchheim-Teck, Germany; b.hellmich{at}


Background Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations.

Methods Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations.

Results Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40–60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons.

Conclusions We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.

  • large vessel vasculitis
  • giant cell arteritis
  • Takayasu arteritis
  • management
  • Eular recommendations
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  • Handling editor Josef S Smolen

  • Correction notice This article has been corrected since it published Online First. The third affiliations has been updated and the author's names for Maria C Cid and Wolfgang Schmidt have been corrected.

  • Contributors AA and SM conducted the SLR. RL provided substantial methodological advice. BH drafted the first version of the manuscript and subsequent revisions. All authors were involved in the formulation and discussion of the recommendations, reviewed the manuscript and made extensive comments and changes to it. The final version of the manuscript was approved by all authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests EB received consultancies from Roche (payed to the University Medical Center Groningen). FB received grants from Horizon and Mundipharma and speaker fees and/or consultancies from Horizon, Mundipharma, Roche and Sanofi. HB received a grant and speaker fees from Roche. BD received consultancies and/or speaker fees from BMS, Chugai, GSK and Roche. CD received and grant from Celgene and speaker fees and/or consultancies from Abbvie, BMS, Lilly, MSD, Pfizer, Novartis, UCB, Roche and Sanofi. BH received speaker fees and/or consultancies from Abbvie, Boehringer, Chugai, Celgene, MSD, Pfizer, Novartis and Roche. SM received speaker fees and consultancies from Roche and Chugai. WS received a grant from Roche and speaker fees and consultancies from Chugai, GSG, Novartis, Roche and Sanofi. CT received a grant from BMS and speaker fees and/or consultancies from Abbvie, BMS, Pfizer and Roche. PV received a grant for conducting an RCT in GCA from Roche. All other authors have no competing interests.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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