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
- Eular recommendations
Statistics from Altmetric.com
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.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.