George Bruyn

Greta Carrara

Maria Antonietta D’Agostino

Stavros Chrysidis

Bhaskar Dasgupta

Christian Dejaco

Eugenio de Miguel

Andreas Diamantopoulos

Christina Duftner

Ulrich Fredberg

Petra Hanova

Ib Toender Hansen

Wolfgang Hartung

Alojzija Hocevar

Annamaria Iagnocco

Aaron Juche

Tanaz Kermani

Minna Kohler

Matthew Koster

Tove Lorenzen

Raashid Luqmani

Pierluigi Macchioni

Marcin Milchert

Uffe Møller Døhn

Sara Monti Pavia

Chetan Mukhtyar

Esperanza Naredo

Berit Dalsgaard Nielsen

Cristina Ponte

Naina Rastalsky

Sofia Ramiro

Valentin Schäfer

Wolfgang Schmidt

Carlo Scire

Lene Terslev

Kenneth Warrington


























Large Vessel Vasculitis

Working group OMERACT > ANCA Vasculitis (Core Set), Large Vessel Vasculitis and Polymyalgia Rheumatica



Annamaria Iagnocco

Objective of the subgroups work

  • Creating definitions for ultrasound morphology and pathology in suspected large-vessel vasculitis (LVV) which can be applied in future prospective studies.

  • Assessing reliability for these definitions in images, videos and patients with giant cell arteritis (GCA) and in controls

  • Assessing ultrasound as an outcome measurement in giant cell arteritis, possibly also in Takayasu arteritis (TAK)

What is the contribution of the subgroup work to the OMERACT work?

LVV includes GCA and TAK. Outcome in GCA largely relies on patient history and CRP / ESR. With the approval of tocilizumab for the treatment of GCA, follow-up has become more challenging, as IL-6 inhibition itself induces profound reduction of inflammatory markers which make them useless for deciding if a patient is flaring. In this situation it is important to investigate ultrasound as an outcome measurement both for damage and disease activity. In TAK, imaging has already gained greater importance for disease follow-up. However, scarce scientific data has been published so far, particularly on ultrasound.


Description of the previous work

  • Meta-analysis for generating definitions for ultrasound in LVV (Chrysidis S, et al. RMD Open. 2018 May 17;4(1):e000598.)

  • Meta-analysis on imaging including ultrasound in diagnosis, outcome prediction and monitoring of LVV (Duftner C, et al. RMD Open. 2018 Feb 2;4(1):e000612.)

  • Reliability assessment of videos and images of temporal and axillary arteries from GCA patients and controls. Kappa values for intra-and inter-reliabilities were >0.8 (Chrysidis S, et al. RMD Open. 2018 May 17;4(1):e000598.)

  • Reliability assessment in GCA patients and controls by experienced sonographers. Kappa values for the diagnosis of GCA after 6 hours training with the ultrasound machines and its settings and examination time of 15-20 minutes for temporal and axillary arteries were 0.76-0.86 for inter-reader reliability and 0.91 for intra-reader reliability. (Schäfer VS, et al. J Rheumatol in press)

  • This work helped to develop Eular Recommendations on Imaging in LVV (Dejaco C, et al. Ann Rheum Dis. 2018 May;77(5):636-643.)

  • Artery wall thickness will be tested as an outcome parameter in future studies. Two studies have determined cut-off values for temporal arteries, one of these studies also for axillary and facial arteries (Schäfer VS, et al. Rheumatology (Oxford). 2017 Sep 1;56(9):1479-1483 and Miguel E, et al. Rheumatology (Oxford). 2018 Feb 1;57(2):318-321.)

  • A study testing the definitions created by the group as an outcome parameter started as part of the phase 3 SIRRESTA trial (IL-6 inhibitor sirukumab versus placebo in GCA). Sonographers were trained and certified in Oxford in 2016. Patients were recruited, videos were saved and reviewed in 2017. Unfortunately, the sponsor, GlaxoSmithKline, prematurely terminated the trial in October 2017 based on the decision to discontinue development of sirukumab in autoimmune diseases.


Reporting back of work done since ACR 2017

  • An investigator initiated prospective pilot study (PROTEA) to test the ultrasound definitions and to measure wall thickness of temporal and axillary arteries started recruiting patients in Lisbon (Cristina Ponte) and in Pavia (Sara Monti), and will start recruiting patients in Oxford (Cláudio Pereira / Raashid Luqmani) after local ethics approval.

  • Three rounds of a Delphi exercise for creating definitions of chronic wall changes of axillary arteries have been done. At least one more Delphi round needs to be done before definitions can be agreed on (Valentin Schäfer, Christian Dejaco).


Next steps and discussion points

  • Testing ultrasound definitions for LVV in another prospective phase 2 and 3 pharmaceutical trial, currently searching sponsors who could include such an ultrasound substudy.

  • Defining elementary ultrasound lesions in polymyalgia rheumatica (PMR). Dario Camellino (Genua, Italy) and Valentin Schäfer will do a systemic literature research which will be followed by a Delphi exercise.

  • The EULAR / ACR classification criteria for PMR include ultrasound of shoulders and hips. They are preliminary as they have not been tested in a separate patient population. The group aims at initiating a prospective international multi-centre study for validating the criteria. This study should also include arterial ultrasound to estimate the prevalence of GCA at baseline and follow-up (Bhaskar Dasgupta).