The 2023 ACR/EULAR classification criteria for calcium pyrophosphate deposition disease

Abhishek, Abhishek; Tedeschi, Sara K.; Pascart, Tristan; Latourte, Augustin; Dalbeth, Nicola; Neogi, Tuhina; Fuller, Amy; Rosenthal, Ann; Becce, Fabio; Bardin, Thomas; Ea, Hang Korng; Filippou, Georgios; Fitzgerald, John; Iagnocco, Annamaria; Lioté, Frédéric; McCarthy, Geraldine M.; Ramonda, Roberta; Richette, Pascal; Sivera, Francisca; Andres, Mariano; Cipolletta, Edoardo; Doherty, Michael; Pascual, Eliseo; Perez-Ruiz, Fernando; So, Alexander; Jansen, Tim L.; Kohler, Minna J.; Stamp, Lisa K.; Yinh, Janeth; Adinolfi, Antonella; Arad, Uri; Aung, Thanda; Benillouche, Eva; Bortoluzzi, Alessandra; Dau, Jonathan; Maningding, Ernest; Fang, Meika; Figus, Fabiana; Filippucci, Emilio; Haslett, Janine; Janssen, Matthijs; Kaldas, Marian; Kimoto, Maryann; Leamy, Kelly; Navarro, Geraldine M.; Sarzi-Puttini, Piercarlo; Scirè, Carlo; Silvagni, Ettore; Sirotti, Silvia; Stack, John; Truong, Linh; Xie, Chen; Yokose, Chio; Hendry, Alison; Terkeltaub, Robert; Taylor, William J.; Choi, Hyon K.

Abstract

Objective Calcium pyrophosphate deposition (CPPD) disease is prevalent and has diverse presentations, but there are no validated classification criteria for this symptomatic arthritis. The American College of Rheumatology (ACR) and EULAR have developed the first-ever validated classification criteria for symptomatic CPPD disease.

Methods Supported by the ACR and EULAR, a multinational group of investigators followed established methodology to develop these disease classification criteria. The group generated lists of candidate items and refined their definitions, collected de-identified patient profiles, evaluated strengths of associations between candidate items and CPPD disease, developed a classification criteria framework, and used multi-criterion decision analysis to define criteria weights and a classification threshold score. The criteria were validated in an independent cohort.

Results Among patients with joint pain, swelling, or tenderness (entry criterion) whose symptoms are not fully explained by an alternative disease (exclusion criterion), the presence of crowned dens syndrome or calcium pyrophosphate crystals in synovial fluid are sufficient to classify a patient as having CPPD disease. In the absence of these findings, a score>56 points using weighted criteria, comprising clinical features, associated metabolic disorders, and results of laboratory and imaging investigations, can be used to classify as CPPD disease. These criteria had a sensitivity of 92.2% and specificity of 87.9% in the derivation cohort (190 CPPD cases, 148 mimickers), whereas sensitivity was 99.2% and specificity was 92.5% in the validation cohort (251 CPPD cases, 162 mimickers).

Conclusion The 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field.

Citation

Abhishek, A., Tedeschi, S. K., Pascart, T., Latourte, A., Dalbeth, N., Neogi, T., …Choi, H. K. (2023). The 2023 ACR/EULAR classification criteria for calcium pyrophosphate deposition disease. Annals of the Rheumatic Diseases, 82(10), 1248-1257. https://doi.org/10.1136/ard-2023-224575

• Journal Article Type: Article
• Acceptance Date: Jun 14, 2023
• Online Publication Date: Jul 26, 2023
• Publication Date: Sep 19, 2023
• Deposit Date: Jul 26, 2023
• Publicly Available Date: Jul 26, 2023
• Journal: Annals of the Rheumatic Diseases
• Print ISSN: 0003-4967
• Electronic ISSN: 1468-2060
• Publisher: BMJ Publishing Group
• Peer Reviewed: Peer Reviewed
• Volume: 82
• Issue: 10
• Pages: 1248-1257
• DOI: https://doi.org/10.1136/ard-2023-224575
• Keywords: General Biochemistry, Genetics and Molecular Biology; Immunology; Immunology and Allergy; Rheumatology
• Public URL: https://nottingham-repository.worktribe.com/output/23479529
• Publisher URL: https://ard.bmj.com/content/82/10/1248