2018 Update of the EULAR recommendations for the management of large vessel vasculitis

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift


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.


  • Bernhard Hellmich
  • Ana Agueda
  • Sara Monti
  • Frank Buttgereit
  • Hubert De Boysson
  • Elisabeth Brouwer
  • Rebecca Cassie
  • Maria Cinta Cid
  • Bhaskar Dasgupta
  • Christian Dejaco
  • Gulen Hatemi
  • Nicole Hollinger
  • Alfred Mahr
  • Susan P. Mollan
  • Chetan Mukhtyar
  • Cristina Ponte
  • Carlo Salvarani
  • Rajappa Sivakumar
  • Xinping Tian
  • Gunnar Tomasson
  • Wofgang Schmidt
  • Peter M. Villiger
  • Richard Watts
  • Chris Young
  • Raashid Ahmed Luqmani
Enheter & grupper
Externa organisationer
  • University of Tübingen
  • University of Pavia
  • Charité Universitätsmedizin Berlin
  • University Medical Center Groningen
  • University of Barcelona
  • Medical University of Graz
  • Paris Diderot University
  • University Hospitals Birmingham
  • University of Birmingham
  • Norfolk and Norwich University Hospital NHS Trust
  • University of Lisbon
  • Arcispedale Santa Maria Nuova
  • Peking Union Medical College
  • University of Iceland
  • Skåne University Hospital
  • Bern University Hospital
  • University of Oxford
  • Centro Hospitalar do Baixo Vouga
  • Centre Hospitalier Universitaire Côte de Nacre
  • No affiliation available (private)
  • Southend Hospital
  • Istanbul University
  • Instituto de Medicina Molecular, Lisboa
  • GLB Hospitals and Acute Stroke Centers
  • Immanuel Hospital Berlin
  • University of East Anglia

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Reumatologi och inflammation


Sidor (från-till)19-30
TidskriftAnnals of the Rheumatic Diseases
Utgåva nummer1
Tidigt onlinedatum2019 jul 3
StatusPublished - 2020
Peer review utfördJa