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

• Published in: Annals of the rheumatic diseases Vol. 79; no. 1; pp. 19 - 30
• Main Authors: Hellmich, Bernhard, Agueda, Ana, Monti, Sara, Buttgereit, Frank, de Boysson, Hubert, Brouwer, Elisabeth, Cassie, Rebecca, Cid, Maria C, Dasgupta, Bhaskar, Dejaco, Christian, Hatemi, Gulen, Hollinger, Nicole, Mahr, Alfred, Mollan, Susan P, Mukhtyar, Chetan, Ponte, Cristina, Salvarani, Carlo, Sivakumar, Rajappa, Tian, Xinping, Tomasson, Gunnar, Turesson, Carl, Schmidt, Wolfgang, Villiger, Peter M, Watts, Richard, Young, Chris, Luqmani, Raashid Ahmed
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd and European League Against Rheumatism 01.01.2020; Elsevier Limited
• Subjects: Antibodies, Monoclonal, Humanized - therapeutic use; Anticoagulants; Antirheumatic Agents - therapeutic use; Aortitis - diagnostic imaging; Aortitis - drug therapy; Aortitis - pathology; Arteritis; Autoimmune diseases; Biopsy; Clinical Medicine; Clinical trials; Eular recommendations; giant cell arteritis; Giant Cell Arteritis - diagnostic imaging; Giant Cell Arteritis - drug therapy; Giant Cell Arteritis - pathology; Glucocorticoids; Glucocorticoids - therapeutic use; Humans; Immunomodulators; Klinisk medicin; large vessel vasculitis; Literature reviews; Management; Medical and Health Sciences; Medicin och hälsovetenskap; Methotrexate; Methotrexate - therapeutic use; Monoclonal antibodies; Patients; Prednisone; Recommendation; Remission; Stroke; Systematic review; Takayasu arteritis; Takayasu Arteritis - diagnostic imaging; Takayasu Arteritis - drug therapy; Takayasu Arteritis - pathology; Takayasu's disease; Task forces; Vasculitis; Vein & artery diseases; Veins & arteries; giant cell arteritis; large vessel vasculitis; Takayasu arteritis; management; Eular recommendations
• ISSN: 0003-4967; 1468-2060; 1468-2060
• DOI: 10.1136/annrheumdis-2019-215672

BackgroundSince 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.MethodsUsing 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.ResultsThree 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.ConclusionsWe 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.