The 2023 ACR/EULAR Classification Criteria for Calcium Pyrophosphate Deposition Disease
Abhishek Abhishek, Sara K. Tedeschi, Tristan Pascart, Augustin Latourte, Nicola Dalbeth, Tuhina Neogi, Amy Fuller, Ann Rosenthal, Fabio Becce, Thomas Bardin, Hang Korng Ea, Georgios Filippou, John FitzGerald, AnnaMaria Iagnocco, Frédéric Lioté, Geraldine M. McCarthy, Roberta Ramonda, Pascal Richette, Francisca Sivera, Mariano Andres, Edoardo Cipolletta, Michael Doherty, Eliseo Pascual, Fernando Perez‐Ruiz, Alexander So, Tim L. Jansen, Minna J. Kohler, Lisa K. Stamp, Janeth Yinh, Antonella Adinolfi, Uri Arad, Thanda Aung, Eva Benillouche, Alessandra Bortoluzzi, Jonathan Dau, Ernest Maningding, Meika A. Fang, Fabiana A. Figus, Emilio Filippucci, Janine Haslett, Matthijs Janssen, Marian Kaldas, Maryann Kimoto, Kelly Leamy, Geraldine M. Navarro, Piercarlo Sarzi‐Puttini, Carlo Scirè, Ettore Silvagni, Silvia Sirotti, John R. Stack, Linh Truong, Chen Xie, Chio Yokose, Alison M. Hendry, Robert Terkeltaub, William J. Taylor, Hyon K. Choi - Immunology
- Rheumatology
- Immunology and Allergy
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.