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Peer-reviewedCC BY-NC 4.0RMD Open

Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises

16 more authors
32 affiliations
  1. 1Department of Rheumatology, Hospital of Brunico (SABES-ASDAA), Teaching Hospital of the Paracelsus Medical University Innsbruck, Bruneck, Italy
  2. 2Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy
  3. 3Rheumatology, Waldfriede Hospital, Berlin, Germany
  4. 4Rheumatology Department, ULS Santa Maria, Centro Académico de Medicina de Lisboa, Lisbon, Portugal
  5. 5Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisboa, Portugal
  6. 6Division of Rheumatology, Medical University of Vienna, Vienna, Austria
  7. 7Department of Rheumatology, Hospital Lillebaelt, Vejle, Denmark
  8. 8Rheumatology Department, Hospital Universitario La Paz, Madrid, Spain
  9. 9Department of Infectious Diseases, Akershus University Hospital, Lørenskog, Norway
  10. 10Department of Rheumatology, Institute of Rheumatology, Prague, Czech Republic
  11. 11Medical Faculty, University of Ljubljana, Ljubljana, Slovenia
  12. 12UMC Ljubljana, Ljubljana, Slovenia
  13. 13Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, India
  14. 14IRCCS Istituto Auxologico Italiano, Milan, Italy
  15. 15Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi, Milan, Italy
  16. 16Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark
  17. 17Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
  18. 18Department of Medicine, The Regional Hospital Horsens, Horsens, Denmark
  19. 19Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
  20. 20Clinic of Internal Medicine III, Oncology, Hematology, Rheumatology and Clinical Immunology, University Hospital Bonn, Bonn, Germany
  21. 21Department of Rheumatology and Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
  22. 22Department of Angiology, University Hospital Basel, Basel, Switzerland
  23. 23Advanced Imaging for Personalized Medicine, Experimental Imaging Centre, IRCCS San Raffaele Scientific Institute, Milan, Italy
  24. 24School of Medicine, Università Vita-Salute San Raffaele, Milan, Italy
  25. 25Department of Radiology, Medical University Innsbruck/Tirol Kliniken GmbH, Innsbruck, Austria
  26. 26Rheumatology Department, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
  27. 27Università Cattolica del Sacro Cuore, Rome, Italy
  28. 28Department of Rheumatology, IRCCS Galeazzi—Sant'Ambrogio Hospital, Milan, Italy
  29. 29Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy
  30. 30Division of Rheumatology, Instituto Nacional de Rehabilitación Luis Guillermo Ibarra Ibarra, Mexico City, Mexico
  31. 31Rheumatology, Clinical Division of Internal Medicine II, Medical University Innsbruck, Innsbruck, Austria
  32. 32Department of Rheumatology and Immunology, Medical University of Graz, Graz, Austria

Abstract

Objective To evaluate the inter-rater and intrarater reliability of consensus-based definitions of elementary ultrasound lesions in Takayasu arteritis (TAK) in live patient-based reliability exercises.Methods Two live exercises with two rounds each were conducted, each involving 12 vascular sonographers who independently examined five patients with TAK and one control subject. Bilateral common carotid, subclavian and axillary arteries and the abdominal aorta were investigated to identify key elementary ultrasound lesions in TAK: ‘macaroni sign’, stenosis and occlusion. Inter-rater and intrarater reliability were calculated using Fleiss’ and Cohen’s kappa (κ), respectively.Results Overall, inter-rater reliability across the two rounds of both exercises was moderate to good for the ‘macaroni sign’ (κ 0.51-0.74 across the two rounds of both exercises), fair for stenosis (0.24–0.38) and variable for occlusion (0.02–0.60). Intrarater reliability across the two exercises was good to excellent for the ‘macaroni sign’ (0.74–0.81) and moderate for stenosis (0.55–0.64) and occlusion (0.64–1.00; the latter based on a limited number of assessments). In both exercises, inter-rater reliability was good to excellent for the ‘macaroni sign’ in carotids but worse across other vessels and lesion types; intrarater reliability was good to excellent when analysed per single vessel and lesion though limited for occlusions and for the abdominal aorta.Conclusion Among the ultrasound elementary lesions for TAK, the ‘macaroni sign’ revealed moderate to good reliability, while the reproducibility of stenosis and occlusion was worse and more variable. These results provide the methodological basis to develop a new global Outcome Measures in Rheumatology (OMERACT) ultrasound score in TAK.

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BibTeX
@article{lernia2026assessing,
  title = {{Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises}},
  author = {Michele di Lernia and Alessandro Tomelleri and Wolfgang A Schmidt and Milena Bond and Cristina Ponte and Peter Mandl and Stavros Chrysidis and Eugenio De Miguel and Andreas P Diamantopoulos and Petra Hanova and Alojzija Hocevar and Durga Prasanna Misra and Sara Monti and Berit Dalsgaard Nielsen and Niccolò Possemato and Valentin Sebastian Schäfer and Luca Seitz and Pascal Seitz and Markus Aschwanden and Davide Vignale and Antonio Esposito and Gerlig Widmann and Maria-Antonietta D’Agostino and Georgios Filippou and Carlos Pineda and Christina Duftner and Christian Dejaco and on behalf of the Large-Vessel Vasculitis-Ultrasound Subgroup of the OMERACT Ultrasound Working Group},
  journal = {RMD Open},
  year = {2026},
  month = oct,
  doi = {10.1136/rmdopen-2026-006884},
  url = {https://doi.org/10.1136/rmdopen-2026-006884},
  note = {CC BY-NC 4.0},
}
RIS
TY  - JOUR
TI  - Assessing vasculitis in Takayasu arteritis by ultrasound: results of OMERACT patient-based reliability exercises
AU  - Michele di Lernia
AU  - Alessandro Tomelleri
AU  - Wolfgang A Schmidt
AU  - Milena Bond
AU  - Cristina Ponte
AU  - Peter Mandl
AU  - Stavros Chrysidis
AU  - Eugenio De Miguel
AU  - Andreas P Diamantopoulos
AU  - Petra Hanova
AU  - Alojzija Hocevar
AU  - Durga Prasanna Misra
AU  - Sara Monti
AU  - Berit Dalsgaard Nielsen
AU  - Niccolò Possemato
AU  - Valentin Sebastian Schäfer
AU  - Luca Seitz
AU  - Pascal Seitz
AU  - Markus Aschwanden
AU  - Davide Vignale
AU  - Antonio Esposito
AU  - Gerlig Widmann
AU  - Maria-Antonietta D’Agostino
AU  - Georgios Filippou
AU  - Carlos Pineda
AU  - Christina Duftner
AU  - Christian Dejaco
AU  - on behalf of the Large-Vessel Vasculitis-Ultrasound Subgroup of the OMERACT Ultrasound Working Group
PY  - 2026
DA  - 2026/10/30
JO  - RMD Open
DO  - 10.1136/rmdopen-2026-006884
UR  - https://doi.org/10.1136/rmdopen-2026-006884
AB  - Objective To evaluate the inter-rater and intrarater reliability of consensus-based definitions of elementary ultrasound lesions in Takayasu arteritis (TAK) in live patient-based reliability exercises.Methods Two live exercises with two rounds each were conducted, each involving 12 vascular sonographers who independently examined five patients with TAK and one control subject. Bilateral common carotid, subclavian and axillary arteries and the abdominal aorta were investigated to identify key elementary ultrasound lesions in TAK: ‘macaroni sign’, stenosis and occlusion. Inter-rater and intrarater reliability were calculated using Fleiss’ and Cohen’s kappa (κ), respectively.Results Overall, inter-rater reliability across the two rounds of both exercises was moderate to good for the ‘macaroni sign’ (κ 0.51-0.74 across the two rounds of both exercises), fair for stenosis (0.24–0.38) and variable for occlusion (0.02–0.60). Intrarater reliability across the two exercises was good to excellent for the ‘macaroni sign’ (0.74–0.81) and moderate for stenosis (0.55–0.64) and occlusion (0.64–1.00; the latter based on a limited number of assessments). In both exercises, inter-rater reliability was good to excellent for the ‘macaroni sign’ in carotids but worse across other vessels and lesion types; intrarater reliability was good to excellent when analysed per single vessel and lesion though limited for occlusions and for the abdominal aorta.Conclusion Among the ultrasound elementary lesions for TAK, the ‘macaroni sign’ revealed moderate to good reliability, while the reproducibility of stenosis and occlusion was worse and more variable. These results provide the methodological basis to develop a new global Outcome Measures in Rheumatology (OMERACT) ultrasound score in TAK.
SN  - 2056-5933
ER  -