Large Vessel Vasculitis

TARGET DOMAIN:

For GCA: Vasculitis (temporal and axillary arteries)

For PMR: Tenosynovitis (biceps tendon), effusion / synovitis (glenohumeral joint, hip joint), bursitis (deltoid, trochanteric)

MENTOR:

L TERSLEV  

Convenors: Wolfgang A. Schmidt, Christian Dejaco, Valentin S. Schäfer

Website responsible: C. Dejaco

Objective of the subgroups work: 

• Define domain components for cranial arteries, extra-cranial arteries, chronic vasculitis

• Define target domains and domain components / elementary lesions for polymyalgia rheumatica (PMR)

• Test reliabilities of definitions on still images, videos, and patients

Description of the possible contribution of the subgroup work for OMERACT

· Develop an “OMERACT halo score” for structural pathology (intima-media thickness) as an outcome measure tool for studies.

· Develop a “PMR” score as an outcome measure tool for studies.

· Apply these scores in prospective cohorts and in trials.

Short description of the previous work (including publications)

• Chrysidis S, Duftner C, Dejaco C, Schäfer VS, Ramiro S, Carrara G, Scirè CA, Hocevar A, Diamantopoulos AP, Iagnocco A, Mukhtyar C, Ponte C, Naredo E, De Miguel E, Bruyn GA, Warrington KJ, Terslev L, Milchert M, D'Agostino MA, Koster MJ, Rastalsky N, Hanova P, Macchioni P, Kermani TA, Lorenzen T, Døhn UM, Fredberg U, Hartung W, Dasgupta B, Schmidt WA. Definitions and reliability assessment of elementary ultrasound lesions in giant cell arteritis: a study from the OMERACT Large Vessel Vasculitis Ultrasound Working Group. RMD Open 2018;4:e000598. Publication on definitions and image / video reliability.

• Schäfer VS, Chrysidis S, Dejaco C, Duftner C, Iagnocco A, Bruyn GA, Carrara G, D'Agostino MA, De Miguel E, Diamantopoulos AP, Fredberg U, Hartung W, Hocevar A, Juche A, Kermani TA, Koster MJ, Lorenzen T, Macchioni P, Milchert M, Døhn UM, Mukhtyar C, Ponte C, Ramiro S, Scirè CA, Terslev L, Warrington KJ, Dasgupta B, Schmidt WA. Assessing Vasculitis in Giant Cell Arteritis by Ultrasound: Results of OMERACT Patient-based Reliability Exercises. J Rheumatol 2018;45:1289-1295. doi: 10.3899/jrheum.171428. Epub 2018 Jul 1. Publication on two patient-based reliability exercises.

• Schäfer VS, Juche A, Ramiro S, Krause A, Schmidt WA. Ultrasound cut-off values for intima-media thickness of temporal, facial and axillary arteries in giant cell arteritis. Rheumatology (Oxford) 2017;56:1479-83. This study was performed for creating the basis for a quantitative OMERACT ultrasound score based on the intima-media thickness of temporal and axillary arteries.

• Schäfer VS, Chrysidis S, Schmidt WA, Duftner C, Iagnocco A, Bruyn GA, Carrara G, De Miguel E, Diamantopoulos AP, Nielsen BD, Fredberg U, Hartung W, Hanova P, Hansen IT, Hocevar A, Juche A, Kermani TA, Lorenzen T, Macchioni P, Milchert M, Døhn UM, Mukhtyar C, Monti S, Ponte C, Seitz L, Scirè CA, Terslev L, Dasgupta B, Keen HI, Pineda C, Dejaco C. Semin Arthritis Rheum 2021;51:951-6. OMERACT definition and reliability assessment of chronic ultrasound lesions of the axillary artery in giant cell arteritis.

 Reporting back of work done since previous F2F meeting (ACR, EULAR or OMERACT)

• The Delphi exercise for creating an OMERACT halo score for GCA has been completed by June 18, 2022. The results will be soon announced.

• In parallel to the Delphi exercise, the sensitivity to change for several score options was calculated by Carlo Scire and colleagues on a prospective cohort (PROTEA study; Ponte C, et al. Ann Rheum Dis 2021;80:1475-82). The group members were informed on the results during the Delphi exercise.

• Further, the inter-and intra-reader reliability of temporal and axillary intima-media thickness (IMT) of GCA patients and persons without GCA was tested on DICOM images of temporal and axillary arteries. The reliability was also tested for several score options, and the members were informed on the results during the Delphi exercise.

• Further input came from the experience with the GUSTO study, another prospective study, in which IMT measurements of temporal and axillary arteries was performed (Seitz L, et al. Rheumatology 2021;60:5052-9).

• The manuscript for describing the Delphi exercise including the reliability exercise is currently being prepared by Christian Dejaco.

• A systematic literature research (SLR) for PMR following an older SLR (Mackie SL, et al. RMD Open 2015;1:e000100) was done, and elementary lesions in PMR were defined in a Delphi exercise.

Next steps and discussion points (if applicable)

• The new OMERACT halo score will now be tested in other prospective cohorts of several members of the group. This will also include the prospective cohort of the TitAIN trial (phase 2 trial on secukinumab versus placebo: Venhoff N, et al ACR Meeting 2021, Abstract L19; EULAR Congress 2022, Abstract OP0182; Venhoff N, et al. Trials 2021;22:543).

• The new OMERACT halo score will also be tested in the GigAINt trial, a prospective phase 3 study to compare secukinumab with placebo in GCA.

• Further work on definitions and reliability needs to be done in PMR. As 5 elementary lesions have been defined, at least a lesion count (0-10) can already be performed in studies.

  GCA Phantom Project: 3D-printed vessels have been developed for teaching. They are embedded in a special gelantine. These vessels mimic a halo sign or normal temporal and axillary arteries and allow for IMT measurement. Currently, these phantoms are sent around among group members for a reliability exercise.