Classification Criteria for Axial Disease in Youth with Juvenile Spondyloarthritis

Pamela F Weiss; Timothy G Brandon; Amita Aggarwal; Ruben Burgos-Vargas; Robert A Colbert; Gerd Horneff; Ronald M Laxer; Kirsten Minden; Angelo Ravelli; Nicolino Ruperto; Judith A Smith; Matthew L Stoll; Shirley M Tse; Filip Van den Bosch; Walter P Maksymowych; Robert G Lambert; David M Biko; Nancy A Chauvin; Michael L Francavilla; Jacob L Jaremko; Nele Herregods; Ozgur Kasapcopur; Mehmet Yildiz; Hemalatha Srinivasalu; Daniel J Lovell; Peter A Nigrovic; Ivan Foeldvari; Marisa S Klein-Gitelman; Seza Ozen; Ray Naden; Alison M Hendry; Rik Joos

doi:10.1002/art.42959

Classification Criteria for Axial Disease in Youth with Juvenile Spondyloarthritis

Arthritis Rheumatol. 2024 Jul 22. doi: 10.1002/art.42959. Online ahead of print.

Authors

Pamela F Weiss¹, Timothy G Brandon², Amita Aggarwal³, Ruben Burgos-Vargas⁴, Robert A Colbert⁵, Gerd Horneff^{6

7}, Ronald M Laxer⁸, Kirsten Minden^{9

10}, Angelo Ravelli¹¹, Nicolino Ruperto¹², Judith A Smith¹³, Matthew L Stoll¹⁴, Shirley M Tse¹⁵, Filip Van den Bosch¹⁶, Walter P Maksymowych¹⁷, Robert G Lambert¹⁸, David M Biko¹⁹, Nancy A Chauvin²⁰, Michael L Francavilla²¹, Jacob L Jaremko¹⁸, Nele Herregods²², Ozgur Kasapcopur²³, Mehmet Yildiz²³, Hemalatha Srinivasalu^{24

25}, Daniel J Lovell²⁶, Peter A Nigrovic^{27

28}, Ivan Foeldvari²⁹, Marisa S Klein-Gitelman³⁰, Seza Ozen³¹, Ray Naden^#³², Alison M Hendry³³, Rik Joos³⁴

Affiliations

¹ Department of Pediatrics, Division of Rheumatology and Clinical Futures, Children's Hospital of Philadelphia and Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
² Department of Pediatrics, Division of Rheumatology and Clinical Futures, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
³ Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
⁴ Department of Rheumatology, Hospital General de Mexico Dr Eduardo Ligeaga, Mexico.
⁵ National Institute of Arthritis, Musculoskeletal, and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
⁶ Department of General Paediatrics, Asklepios Klinik Sankt Augustin, Sankt Augustin, Germany.
⁷ Department of Pediatric and Adolescent Medicine, University Hospital Cologne, Cologne, Germany.
⁸ Professor of Pediatrics and Medicine, University of Toronto, Staff Rheumatologist, The Hospital for Sick Children and St. Michael's Hospital.
⁹ 1- Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt- Universität zu Berlin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany.
¹⁰ 2 - Deutsches Rheuma-Forschungszentrum Berlin, Germany.
¹¹ Direzione Scientifica, IRCCS Istituto Giannina Gaslini and Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DINOGMI), Università degli Studi di Genova, Genoa, Italy.
¹² IRCCS Istituto Giannina Gaslini, Gaslini Trial Centre/Servizio di Sperimentazioni Cliniche Pediatriche, PRINTO, Genova, Italy.
¹³ Department of Pediatrics, University of Wisconsin-Madison School of Medicine and Public Health, Madison, WI, USA.
¹⁴ Department of Pediatrics, University of Alabama at Birmingham; Birmingham, AL, USA.
¹⁵ Department of Paediatrics, Division of Rheumatology, The Hospital for Sick Children (SickKids), University of Toronto, Toronto, ON, Canada.
¹⁶ VIB-UGent Center for Inflammation Research, Department of Internal Medicine and Pediatrics, Ghent University Hospital, Ghent, Belgium.
¹⁷ Professor of Medicine, Department of Medicine, University of Alberta and Chief Medical Officer, CARE Arthritis, Edmonton, AB, Canada.
¹⁸ Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, AB, Canada.
¹⁹ Department of Radiology, Children's Hospital of Philadelphia and Department of Radiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
²⁰ Department of Radiology, Penn State Health Milton S. Hershey Children's Hospital, Hershey, PA, USA.
²¹ Department of Radiology, Whiddon College of Medicine, University of South Alabama, Mobile, AL, USA.
²² Department of Radiology and Nuclear Medicine, Ghent University Hospital, Ghent, Belgium.
²³ Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.
²⁴ Division of Rheumatology, Children's National Hospital, George Washington University School of Medicine, Washington, DC, USA.
²⁵ Pediatric Translational Research Branch, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
²⁶ Division of Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
²⁷ Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
²⁸ Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
²⁹ Hamburg Centre for Paediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany.
³⁰ Department of Pediatrics, Northwestern University Feinberg School of Medicine, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
³¹ Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey.
³² Departments of Medicine/Obstetric Medicine, Auckland City Hospital, New Zealand and McMaster University, Hamilton, ON, Canada.
³³ General Medicine and Rheumatology, Division of Medicine, Counties Manukau District Health Board, Auckland, New Zealand.
³⁴ consultant in Pediatric Rheumatology, university hospital Gent, Belgium and head of service Dept of Rheumatology, ZNA, Antwerp.

^# Contributed equally.

PMID: 39039899
DOI: 10.1002/art.42959

Abstract

Objectives: To develop and validate classification criteria for axial disease in youth with juvenile spondyloarthritis (SpA; AxJSpA).

Methods: This international initiative consisted of four phases: 1) Item generation; 2) Item reduction; 3) Criteria development; and 4) Validation of the AxJSpA criteria by an independent team of experts in an internationally representative Validation cohort.

Results: These criteria are intended to be used on youth with a physician diagnosis of juvenile SpA and for whom axial disease is suspected. Item generation consisted of a systematic literature review and a free-listing exercise using input from international physicians and collectively resulted in 108 items. After the item reduction exercise and expert panel input, 37 items remained for further consideration. The final AxJSpA criteria domains included: imaging: active inflammation, imaging: structural lesions, pain chronicity, pain pattern, pain location, stiffness, and genetics. The most heavily weighted domains were active inflammation and structural lesions on imaging. Imaging typical of sacroiliitis was deemed necessary, but not sufficient, to classify a youth with AxJSpA. The threshold for classification of AxJSpA was a score of ≥55 (out of 100). When tested in the validation data set, the final criteria had a specificity of 97.5% (95% CI: 91.4-99.7), sensitivity of 64.3% (95% CI: 54.9-73.1) and Area Under the Receiver Operating Characteristic (AUROC) curve of 0.81 (95% CI: 0.76-0.86).

Conclusions: The new AxJSpA classification criteria require an entry criterion, physician diagnosis of juvenile SpA, and include seven weighted domains. The AxJSpA classification criteria are validated and designed to identify participants for research studies.