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

Published on Jan 1, 2020in Annals of the Rheumatic Diseases14.299
· DOI :10.1136/ANNRHEUMDIS-2019-215672
Bernhard Hellmich35
Estimated H-index: 35
(University of Tübingen),
Ana Filipa Águeda3
Estimated H-index: 3
+ 23 AuthorsRaashid Luqmani53
Estimated H-index: 53
(University of Oxford)
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.
  • References (139)
  • Citations (48)
📖 Papers frequently viewed together
11 Authors (Sara Monti, ..., Bernhard Hellmich)
5 Citations
5 Citations
78% of Scinapse members use related papers. After signing in, all features are FREE.
#1Ana Filipa ÁguedaH-Index: 3
#2Sara Monti (UNIPV: University of Pavia)H-Index: 9
Last. Bernhard Hellmich (University of Tübingen)H-Index: 35
view all 12 authors...
5 CitationsSource
#1Sara MontiH-Index: 9
#2Ana Filipa ÁguedaH-Index: 3
Last. Bernhard HellmichH-Index: 35
view all 11 authors...
5 CitationsSource
Background European League Against Rheumatism (EULAR) recommendations on large vessel vasculitis (LVV) management were published in 2009.1 However, given recent advances, especially in therapeutics, an update was needed. Objectives To collect available evidence on management of LVV to inform the 2018 update of the EULAR management recommendations. Methods Two independent systematic literature reviews (SLRs) were performed, one focused on diagnosis and monitoring and the other on drugs and surgic...
1 CitationsSource
#1John J. McNeil (Florey Institute of Neuroscience and Mental Health)H-Index: 71
#2Rory Wolfe (Florey Institute of Neuroscience and Mental Health)H-Index: 63
Last. Anne M. Murray (Florey Institute of Neuroscience and Mental Health)H-Index: 33
view all 33 authors...
Abstract Background Aspirin is a well-established therapy for the secondary prevention of cardiovascular events. However, its role in the primary prevention of cardiovascular disease is unclear, especially in older persons, who have an increased risk. Methods From 2010 through 2014, we enrolled community-dwelling men and women in Australia and the United States who were 70 years of age or older (or ≥65 years of age among blacks and Hispanics in the United States) and did not have cardiovascular ...
124 CitationsSource
#2Sabine AdlerH-Index: 10
Last. Peter M. VilligerH-Index: 44
view all 8 authors...
Objective. To analyse magnetic resonance angiographic (MRA) vessel wall signals from a randomized controlled trial of tocilizumab (TCZ) to treat GCA. Methods. Participants were assigned in a 2:1 ratio to receive either TCZ + glucocorticoids (GCs) or placebo +GC infusions at 4-week intervals for 52 weeks. GCs were started at 1 mg/kg/day, then tapered to 0.1 mg/kg/day at week 12 and thereafter down to zero. Patients with initial positive MRA findings underwent control MRA at weeks 12 and 52. Vesse...
18 CitationsSource
#1Christian DejacoH-Index: 27
#2Sofia Ramiro (LUMC: Leiden University Medical Center)H-Index: 27
Last. Wolfgang A. SchmidtH-Index: 32
view all 20 authors...
114 CitationsSource
#1Yoshikazu NakaokaH-Index: 22
#2Mitsuaki Isobe (Tokyo Medical and Dental University)H-Index: 49
Last. Norihiro Nishimoto (TMU: Tokyo Medical University)H-Index: 53
view all 9 authors...
Objective To investigate the efficacy and safety of the interleukin-6 receptor antibody tocilizumab in patients with Takayasu arteritis (TAK). Methods Patients with TAK who had relapsed within the previous 12 weeks were induced into remission with oral glucocorticoid therapy. In this double-blind, placebo-controlled trial, patients were randomly assigned 1:1 to receive weekly tocilizumab 162 mg or placebo subcutaneously, and oral glucocorticoids were tapered 10 %/week from week 4 to a minimum of...
54 CitationsSource
#1Sara Monti (UNIPV: University of Pavia)H-Index: 9
#2Alberto FlorisH-Index: 8
Last. Raashid Luqmani (NOC: Nuffield Orthopaedic Centre)H-Index: 53
view all 8 authors...
: Colour duplex sonography (CDS) of temporal arteries and large vessels is an emerging diagnostic tool for GCA. CDS can detect wall oedema, known as a halo, throughout the length of the vessel and shows higher sensitivity compared with biopsy. Specificity reaches 100% in case of bilateral halos. A positive compression sign has been demonstrated to be a robust marker with excellent inter-observer agreement. The assessment of other large vessels, particularly the axillary arteries, is recognized t...
24 CitationsSource
#1Christina DuftnerH-Index: 24
#2Christian DejacoH-Index: 27
Last. Sofia Ramiro (LUMC: Leiden University Medical Center)H-Index: 27
view all 6 authors...
Objectives To perform a systematic literature review on imaging techniques for diagnosis, outcome prediction and disease monitoring in large vessel vasculitis (LVV) informing the European League Against Rheumatism recommendations for imaging in LVV. Methods Systematic literature review (until 10 March 2017) of diagnostic and prognostic studies enrolling >20 patients and investigating ultrasound, MRI, CT or positron emission tomography (PET) in patients with suspected and/or established primary L...
30 CitationsSource
Cited By48
#1Fei Kang (Fourth Military Medical University)H-Index: 15
#2Qing Han (Fourth Military Medical University)H-Index: 3
Last. Jing Wang (Fourth Military Medical University)H-Index: 18
view all 11 authors...
PURPOSE To assess the performance of PET vascular activity score (PETVAS) in comparison with SUVmax, inflammatory biomarkers and ITAS-2010 score in a cohort of TAK patients. METHODS Sixty-four PET/CT scans acquired from 54 TAK patients were analyzed. The inflammatory activity was qualitatively determined by physician's global assessment and quantitatively determined by ITAS-2010 score. SUVmax and PETVAS were acquired by consensus review. Levels of the inflammatory biomarkers C-reactive protein (...
Last. Fernando Martínez-ValleH-Index: 7
view all 3 authors...
#1Sun Ying (Fudan University)
#2Cui Xiaomeng (Fudan University)
Last. Jiang Lin-di (Fudan University)
view all 10 authors...
Background:Leflunomide (LEF) has been considered as an alternative treatment for Takayasu arteritis (TA); however, data on its efficacy are still scanty.Objective:To investigate the efficacy and sa...
Resumen Las infecciones, entre ellas las oportunistas, constituyen una causa importante y frecuente de morbilidad y mortalidad en los pacientes con enfermedades reumaticas y autoinmunitarias sistemicas. La neumonia por Pneumocystis jirovecii, clasicamente considerada propia de pacientes con VIH, trasplantados o tratados con quimioterapia oncologica, aparece cada vez con mayor frecuencia en estos pacientes. Es por ello conveniente que los reumatologos conozcan su mecanismo de produccion, manifest...
: Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) both belong to the group of large vessel vasculitides and require long-term drug treatment. Glucocorticoids (GC) are the first choice for the treatment of both diseases. For GCA immunosuppressants, such as tocilizumab or methotrexate should be considered in cases of treatment refractory and relapses or if there is a high risk for GC-related adverse events. In TAK patients the use of immunosuppressive agents should be considered for all ...
: Despite advances in the diagnosis and treatment, the mortality rate of Takayasu arteritis (TAK) is still elevated even today. The diagnosis is often made after a long time delay and the course of the disease is characterized by progressive structural vascular lesions. Recently, new recommendations for the management of large vessel vasculitis were published by the European League Against Rheumatism (EULAR). For induction of remission oral glucocorticoids (GC) are administered in an initial dai...
#1Taotao Li (Capital Medical University)H-Index: 1
Last. Lili PanH-Index: 3
view all 5 authors...
OBJECTIVES: Takayasu arteritis (TA) is a rare granulomatous vasculitis. Numano type V was the most frequently involved arteritis in China. In our study, we aimed to investigate the characteristics of the type V TA in Chinese people. METHODS: We retrospectively reviewed 143 medical charts of TA patients who were admitted to Beijing Anzhen Hospital from 2012 to 2019. All statistical studies were carried out with the SPSS program V.24.0. RESULTS: The duration of disease at first admission of type V...
#1Sema Kaymaz-Tahra (Marmara University)H-Index: 1
#2Fatma Alibaz-Oner (Marmara University)H-Index: 13
Last. Haner Direskeneli (Marmara University)H-Index: 39
view all 3 authors...
ABSTRACT Objective : To assess the progression and the factors associated with damage in Takayasu‘s arteritis (TAK) patients during routine follow-up. Methods : Patients diagnosed with TAK and had >6 months follow-up were enrolled in this study retrospectively. Takayasu's arteritis damage score (TADS) and vasculitis damage index (VDI) were determined at diagnosis and at the end of the follow-up and variables associated with damage scores were assessed. Results : One-hundred fourteen patients (F/...