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. 2024 Dec;76(12):1797-1808.
doi: 10.1002/art.42959. Epub 2024 Sep 8.

Classification Criteria for Axial Disease in Youth With Juvenile Spondyloarthritis

Pamela F Weiss  1 , Timothy G Brandon  2 , Amita Aggarwal  3 , Ruben Burgos-Vargas  4 , Robert A Colbert  5 , Gerd Horneff  6 , Ronald M Laxer  7 , Kirsten Minden  8 , Angelo Ravelli  9 , Nicolino Ruperto  10 , Judith A Smith  11 , Matthew L Stoll  12 , Shirley M Tse  13 , Filip Van den Bosch  14 , Walter P Maksymowych  15 , Robert G Lambert  16 , David M Biko  1 , Nancy A Chauvin  17 , Michael L Francavilla  18 , Jacob L Jaremko  16 , Nele Herregods  14 , Ozgur Kasapcopur  19 , Mehmet Yildiz  19 , Hemalatha Srinivasalu  20 , Daniel J Lovell  21 , Peter A Nigrovic  22 , Ivan Foeldvari  23 , Marisa S Klein-Gitelman  24 , Seza Ozen  25 , Ray Naden, Alison M Hendry  26 , Rik Joos  27
Affiliations

Classification Criteria for Axial Disease in Youth With Juvenile Spondyloarthritis

Pamela F Weiss et al. Arthritis Rheumatol. 2024 Dec.

Abstract

Objective: The goal was to develop and validate classification criteria for axial 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, which 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 the following: imaging of active inflammation, imaging of 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% confidence interval [CI] 91.4%-99.7%), sensitivity of 64.3% (95% CI 54.9%-73.1%), and area under the receiver operating characteristic curve of 0.81 (95% CI 0.76%-0.86%).

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

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Conflict of interest statement

PFW – Royalties/licenses: Up-to-date (<$10K to author); Consulting fees: Site investigator for Pfizer and Abbvie Clinical Trials (Payment to institution), Advisory Board member: Lily, Novartis (all <$10K to author), and Consulting fees: Pfizer (payment to institution); Speaking payment or honoraria: 2022 Rheum Now Speaker (<$5K to author) and Spondyloarthritis Research and Treatment Network – honoraria for educational materials (<$5k to author).

RAC – Leadership or fiduciary role: Childhood Arthritis and Rheumatology Research Alliance.

RML – consulting fees (< 5K to author) Novartis, Sanofi, Eli Lilly; Akros Pharma; Alexion; Royalties/licenses: Up-to-date (<$10K to author)

KM – Speaking payments or honoraria: Novartis, Pfizer, Medac

RGL – Consulting fees: Calyx, CARE Arthritis, Image Analysis Group; Payment or honoraria: Novartis.

OK – Speaking payments or honoraria: Novartis, Abbvie, Pfizer, Roche, Sanofi, Amgen; Leadership or fiduciary role: Chairman of Turkish Pediatric Association.

FVdB – consulting fees Abbvie, Celgene, Eli-Lilly, Galapagos, Janssen, Moonlake, Novartis, Pfizer and UCB.

WPM – Leadership or fiduciary role: Board of Directors of SPARTAN; Other financial or non-financial interests: Chief Medical Officer, CARE Arthritis Limited. Consulting: Abbvie, BMS, Celgene, Eli-Lilly, Galapagos, Novartis, Pfizer, UCB; Speaking: Abbvie, Novartis, Janssen, Pfizer, UCB; Grants: Abbvie, BMS, Eli-Lilly, Novartis, Pfizer, UCB

GH – Speaking payments or honoraria: Novartis, MSD, Pfizer, Roche, Sanofi, Sobi, Biogen

HS – Speaking payment or honoraria: Pfizer (<$5k to author), NIH Intramural Research Program, CARRA Registry Associate, CARRA

PAN - Royalties/licenses: Up-to-date (>$10K to author), American Academy of Pediatrics (<$10k to author); Consulting fees: Apollo, BMS, Century Therapeutics, Exo Therapeutics, Merck, Novartis, Pfizer, Qiagen, Sobi; Grants, BMS, Pfizer; Equity: Edelweiss Immune, Inc.

SO – Consulting fees: SOBI, Novartis, Bayer

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