. 2023 Oct;82(10):1248-1257.

doi: 10.1136/ard-2023-224575. Epub 2023 Jul 26.

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

Abhishek Abhishek^#¹, Sara K Tedeschi^#², Tristan Pascart³, Augustin Latourte⁴, Nicola Dalbeth⁵, Tuhina Neogi⁶, Amy Fuller⁷, Ann Rosenthal⁸, Fabio Becce⁹, Thomas Bardin⁴, Hang-Korng Ea⁴, Georgios Filippou¹⁰, John Fitzgerald^{11

12}, AnnaMaria Iagnocco¹³, Frédéric Lioté^{4

14}, Geraldine M McCarthy^{15

16}, Roberta Ramonda¹⁷, Pascal Richette⁴, Francisca Sivera^{18

19}, Mariano Andrés²⁰, Edoardo Cipolletta²¹, Michael Doherty⁷, Eliseo Pascual²², Fernando Perez-Ruiz²³, Alexander So²⁴, Tim L Jansen^{25

26}, Minna J Kohler²⁷, Lisa K Stamp²⁸, Janeth Yinh²⁷, Antonella Adinolfi²⁹, Uri Arad³⁰, Thanda Aung³¹, Eva Benillouche³², Alessandra Bortoluzzi^{33

34}, Jonathan Dau³⁵, Ernest Maningding³⁶, Meika A Fang^{11

12}, Fabiana A Figus³⁷, Emilio Filippucci²¹, Janine Haslett²⁸, Matthijs Janssen²⁵, Marian Kaldas¹¹, Maryann Kimoto¹¹, Kelly Leamy¹⁶, Geraldine M Navarro³¹, Piercarlo Sarzi-Puttini³⁸, Carlo Scirè³⁹, Ettore Silvagni^{33

34}, Silvia Sirotti⁴⁰, John R Stack^{15

16}, Linh Truong³¹, Chen Xie³¹, Chio Yokose⁴¹, Alison M Hendry⁴², Robert Terkeltaub⁴³, William J Taylor²⁸, Hyon K Choi²⁷

Affiliations

¹ Academic Rheumatology, University of Nottingham, Nottingham, UK abhishek.abhishek@nottingham.ac.uk.
² Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
³ Department of Rheumatology, Lille Catholic University, Saint-Philibert Hospital, Lille, France.
⁴ Université de Paris, INSERM, UMR-S 1132 BIOSCAR, and Service de Rhumatologie, AP-HP, Lariboisière Hospital, Paris, France.
⁵ Department of Medicine, University of Auckland, Auckland, New Zealand.
⁶ Department of Medicine, Section of Rheumatology, Boston University School of Medicine, Boston, Massachusetts, USA.
⁷ Academic Rheumatology, University of Nottingham, Nottingham, UK.
⁸ Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
⁹ Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
¹⁰ Rheumatology Department, IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.
¹¹ David Geffen School of Medicine, University of California, Los Angeles, California, USA.
¹² Veterans Administration for Greater Los Angeles, Los Angeles, California, USA.
¹³ Academic Rheumatology Center, Università degli Studi di Torino, Turin, Italy.
¹⁴ Université Paris Cité, Faculté de Santé, Paris, France.
¹⁵ School of Medicine and Medical Science, University College Dublin, Dublin, Ireland.
¹⁶ Mater Misericordiae University Hospital, Dublin, Ireland.
¹⁷ Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹⁸ Department of Rheumatology, Hospital General Universitario Elda, Elda, Spain.
¹⁹ Department of Clinical Medicine, Universidad Miguel Hernandez, Elche, Spain.
²⁰ Department of Medicine, Rheumatology Section, Hospital General Universitario de Alicante, Universidad Miguel Hernández, Alicante, Spain.
²¹ Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy.
²² Rheumatology Division, Cruces University Hospital, Bilbao, Spain.
²³ Arthritis Investigation Group, Biocruces-Bizkaia Health Research Institute, Spain, Department of Medicine, Medicine and Nursing School, University of the Basque Country, and Basque Country Rheumatology Society, Bilbao, Spain.
²⁴ Lausanne University Hospital, Lausanne, Switzerland.
²⁵ VieCuri Medical Centre, Venlo, The Netherlands.
²⁶ Medical Cell BioPhysics Group, University of Twente, Enschede, The Netherlands.
²⁷ Department of Medicine, Rheumatology Unit, Massachusetts General Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
²⁸ Department of Medicine, University of Otago, Christchurch, New Zealand.
²⁹ Rheumatology Unit, Grande Ospedale Metropolitano Niguarda, Milan, Italy.
³⁰ Department of Rheumatology, Te Whatu Ora-Health New Zealand Waikato, Hamilton, New Zealand.
³¹ Division of Rheumatology, University of California, Los Angeles, California, USA.
³² Department of Rheumatology, Lausanne University Hospital, Lausanne, Switzerland.
³³ Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
³⁴ Azienda Ospedaliera-Universitaria di Ferrara (Cona FE), Cona FE, Italy.
³⁵ Department of Medicine, Rheumatology Unit, Massachusetts General Hospital, Boston, Massachusetts, USA.
³⁶ Highland Hospital, Oakland, California, USA.
³⁷ Rheumatology Division, Local Health Unit (ASL), Turin-3, Collegno and Pinerolo, Italy.
³⁸ Department of Rheumatology, IRCCS Galeazzi-Sant'Ambrogio Hospital, Milan, Italy.
³⁹ Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
⁴⁰ Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy.
⁴¹ Harvard Medical School, Boston, Massachusetts, USA.
⁴² Department of Medicine, General Medicine and Rheumatology, Middlemore Hospital, Counties Manukau Health District, Auckland, New Zealand.
⁴³ San Diego Veterans Administration Healthcare Service, and University of California, San Diego, California, USA.

^# Contributed equally.

PMID: 37495237
PMCID: PMC10529191
DOI: 10.1136/ard-2023-224575

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

Abhishek Abhishek et al. Ann Rheum Dis. 2023 Oct.

. 2023 Oct;82(10):1248-1257.

doi: 10.1136/ard-2023-224575. Epub 2023 Jul 26.

Authors

Affiliations

¹ Academic Rheumatology, University of Nottingham, Nottingham, UK abhishek.abhishek@nottingham.ac.uk.
² Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
³ Department of Rheumatology, Lille Catholic University, Saint-Philibert Hospital, Lille, France.
⁴ Université de Paris, INSERM, UMR-S 1132 BIOSCAR, and Service de Rhumatologie, AP-HP, Lariboisière Hospital, Paris, France.
⁵ Department of Medicine, University of Auckland, Auckland, New Zealand.
⁶ Department of Medicine, Section of Rheumatology, Boston University School of Medicine, Boston, Massachusetts, USA.
⁷ Academic Rheumatology, University of Nottingham, Nottingham, UK.
⁸ Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
⁹ Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
¹⁰ Rheumatology Department, IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.
¹¹ David Geffen School of Medicine, University of California, Los Angeles, California, USA.
¹² Veterans Administration for Greater Los Angeles, Los Angeles, California, USA.
¹³ Academic Rheumatology Center, Università degli Studi di Torino, Turin, Italy.
¹⁴ Université Paris Cité, Faculté de Santé, Paris, France.
¹⁵ School of Medicine and Medical Science, University College Dublin, Dublin, Ireland.
¹⁶ Mater Misericordiae University Hospital, Dublin, Ireland.
¹⁷ Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
¹⁸ Department of Rheumatology, Hospital General Universitario Elda, Elda, Spain.
¹⁹ Department of Clinical Medicine, Universidad Miguel Hernandez, Elche, Spain.
²⁰ Department of Medicine, Rheumatology Section, Hospital General Universitario de Alicante, Universidad Miguel Hernández, Alicante, Spain.
²¹ Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy.
²² Rheumatology Division, Cruces University Hospital, Bilbao, Spain.
²³ Arthritis Investigation Group, Biocruces-Bizkaia Health Research Institute, Spain, Department of Medicine, Medicine and Nursing School, University of the Basque Country, and Basque Country Rheumatology Society, Bilbao, Spain.
²⁴ Lausanne University Hospital, Lausanne, Switzerland.
²⁵ VieCuri Medical Centre, Venlo, The Netherlands.
²⁶ Medical Cell BioPhysics Group, University of Twente, Enschede, The Netherlands.
²⁷ Department of Medicine, Rheumatology Unit, Massachusetts General Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
²⁸ Department of Medicine, University of Otago, Christchurch, New Zealand.
²⁹ Rheumatology Unit, Grande Ospedale Metropolitano Niguarda, Milan, Italy.
³⁰ Department of Rheumatology, Te Whatu Ora-Health New Zealand Waikato, Hamilton, New Zealand.
³¹ Division of Rheumatology, University of California, Los Angeles, California, USA.
³² Department of Rheumatology, Lausanne University Hospital, Lausanne, Switzerland.
³³ Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
³⁴ Azienda Ospedaliera-Universitaria di Ferrara (Cona FE), Cona FE, Italy.
³⁵ Department of Medicine, Rheumatology Unit, Massachusetts General Hospital, Boston, Massachusetts, USA.
³⁶ Highland Hospital, Oakland, California, USA.
³⁷ Rheumatology Division, Local Health Unit (ASL), Turin-3, Collegno and Pinerolo, Italy.
³⁸ Department of Rheumatology, IRCCS Galeazzi-Sant'Ambrogio Hospital, Milan, Italy.
³⁹ Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
⁴⁰ Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy.
⁴¹ Harvard Medical School, Boston, Massachusetts, USA.
⁴² Department of Medicine, General Medicine and Rheumatology, Middlemore Hospital, Counties Manukau Health District, Auckland, New Zealand.
⁴³ San Diego Veterans Administration Healthcare Service, and University of California, San Diego, California, USA.

^# Contributed equally.

PMID: 37495237
PMCID: PMC10529191
DOI: 10.1136/ard-2023-224575

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.

Keywords: Autoimmune Diseases; Immune Complex Diseases; Immune System Diseases.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

**Figure 1:**
Overview of CPPD Classification Criteria development process across the four phases.

**Figure 2:**
Conceptual schematic for applying the CPPD disease classification criteria.

**Figure 3:**
Plot of Steering Committee percentage agreement on classifying patient profiles as CPPD disease for inclusion in a research study (n=8 participating Steering Committee members). The patient profiles were given pseudonyms.

**Figure 4:**
Receiver Operating Characteristic (ROC) curves in derivation cohort (left panel) and validation cohort (right panel) for the patients that were eligible to be scored. In the derivation cohort, data for 60 definite cases and 148 definite mimickers were included. In the validation cohort, data for 65 definite cases and 162 definite mimickers were included.

See this image and copyright information in PMC

References

1. Zhang W, Doherty M, Bardin T, Barskova V, Guerne PA, Jansen TL, et al. European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis. Ann Rheum Dis. 2011;70(4):563–70. - PubMed
1. Salaffi F, De Angelis R, Grassi W. Prevalence of musculoskeletal conditions in an Italian population sample: results of a regional community-based study. I. The MAPPING study. Clinical and experimental rheumatology. 2005;23(6):819–28. - PubMed
1. Ramonda R, Musacchio E, Perissinotto E, Sartori L, Punzi L, Corti M, et al. Prevalence of chondrocalcinosis in Italian subjects from northeastern Italy. The Pro. VA (PROgetto Veneto Anziani) study. Clin Exp Rheumatol. 2009;27(6):981–4. - PubMed
1. Neame R, Carr A, Muir K, Doherty M. UK community prevalence of knee chondrocalcinosis: evidence that correlation with osteoarthritis is through a shared association with osteophyte. Annals of the rheumatic diseases. 2003;62(6):513–8. - PMC - PubMed
1. Felson D, Anderson J, Naimark A, Kannel W, Meenan R. The prevalence of chondrocalcinosis in the elderly and its association with knee osteoarthritis: the Framingham Study. The Journal of rheumatology. 1989;16(9):1241–5. - PubMed

Publication types

Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions

Substances

Actions

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

MeSH terms

Substances

Grants and funding

LinkOut - more resources

Full Text Sources

Medical