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

TLDR

Since the 2009 EULAR recommendations for large vessel vasculitis, new randomized trials and cohort studies have emerged, indicating a need to revise clinical guidance. The task force performed a systematic literature review following EULAR SOPs and consulted 20 experts from 13 countries. The aim was to update the EULAR recommendations for large vessel vasculitis to reflect new evidence and improve patient outcomes. The revised guidelines present ten recommendations, including confirming LVV with imaging or histology, initiating high‑dose glucocorticoids for remission, using tocilizumab or methotrexate as adjuncts, reserving antiplatelet or anticoagulant therapy only when indicated, and providing glucocorticoid‑sparing agents for Takayasu arteritis.

Abstract

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.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.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.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.