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Rheumatology

CPPD

Classification

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

2023 ACR/EULAR Criteria for Calcium Pyrophosphate Deposition Disease

Entry requirement

Ever had at least one episode of joint pain, swelling, or tenderness

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*Episode occurring in a peripheral joint or, in the case of crowned dens syndrome, an axial joint such as C1/C2.

Absolute exclusion criteria

All symptoms are more likely explained by an alternative condition (such as rheumatoid arthritis, gout, psoriatic arthritis, OA, etc.)

Sufficient criteria

Presence of either crowned dens syndrome or synovial fluid analysis demonstrating CPP crystals in a joint with swelling, tenderness, or pain

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Crowned dens syndrome is defined as presence of a) clinical features and b) imaging features. Clinical features include acute or subacute onset of severe pain localised to the upper neck with elevated inflammation markers, limited rotation, and often fever. Mimicking conditions such as polymyalgia rheumatica and meningitis should be excluded. Imaging features include conventional CT showing calcific deposits, typically linear and less dense than cortical bone, in the transverse retro-odontoid ligament (transverse ligament of the atlas), often with an appearance of 2 parallel lines in axial views. Calcifications at the atlanto-axial joint, alar ligament, and/or in pannus adjacent to the tip of the dens are also characteristic. Dual-energy computed tomography (DECT) features include a dual-energy index between 0.016 and 0.036. Both the clinical features and the imaging features must be present. Sufficient criteria are also met if calcium pyrophosphate (CPP) crystals are demonstrated on histopathologic analysis of the joint tissue, provided that the patient is eligible for classification, that is, does not already meet the exclusion criteria. For instance, articular cartilage CPP crystal deposition in patients with end-stage osteoarthritis (OA) cannot be used to classify the patient as having calcium pyrophosphate deposition (CPPD) disease when all symptoms are better explained by the presence of OA (exclusion criteria).

Domains and levels

Age at onset of joint symptoms (pain, swelling, and/or tenderness)

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Time course and symptoms of inflammatory arthritis

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Persistent inflammatory arthritis was defined as ongoing joint swelling with pain and/or warmth in≥1 joint(s). Typical episode was defined as an episode with acute onset or acute worsening of joint pain with swelling and/or warmth that resolves irrespective of treatment.

Sites of typical episode(s) of inflammatory arthritis in peripheral joints

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Related metabolic diseases

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Including hereditary hemochromatosis, primary hyperparathyroidism, hypomagnesemia, Gitelman syndrome, hypophosphatasia, or familial history of CPPD disease.

Synovial fluid crystal analysis from a symptomatic joint

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Synovial fluid analysis should be performed by an individual trained in the use of compensated polarised light microscopy for crystal identification

OA of hand/wrist on imaging (defined as present if the K/L score is ≥2)

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Imaging evidence of CPPD in symptomatic peripheral joint(s)**

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Imaging of at least one symptomatic peripheral joint by CR, US, CT, or DECT is required to be considered for classification if sufficient criteria are not met. Imaging evidence of CPPD refers to calcification of the fibrocartilage or hyaline cartilage. Do not score calcification of the synovial membrane, joint capsule, or tendon. Imaging definitions are published elsewhere. Only consider involvement of peripheral joints.

Number of peripheral joints with evidence of CPPD on any imaging modality regardless of symptoms**

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Statistics

Sensitivity: 92.2%

Specificity: 87.9%

Considerations

The tool is for educational use only, is not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.

Reference

Abhishek A, Tedeschi SK, Pascart T, Latourte A, Dalbeth N, Neogi T, Fuller A, Rosenthal A, Becce F, Bardin T, Ea HK, Filippou G, Fitzgerald J, Iagnocco A, Lioté F, McCarthy GM, Ramonda R, Richette P, Sivera F, Andrés M, Cipolletta E, Doherty M, Pascual E, Perez-Ruiz F, So A, Jansen TL, Kohler MJ, Stamp LK, Yinh J, Adinolfi A, Arad U, Aung T, Benillouche E, Bortoluzzi A, Dau J, Maningding E, Fang MA, Figus FA, Filippucci E, Haslett J, Janssen M, Kaldas M, Kimoto M, Leamy K, Navarro GM, Sarzi-Puttini P, Scirè C, Silvagni E, Sirotti S, Stack JR, Truong L, Xie C, Yokose C, Hendry AM, Terkeltaub R, Taylor WJ, Choi HK. The 2023 ACR/EULAR classification criteria for calcium pyrophosphate deposition disease. Ann Rheum Dis. 2023 Jul 26:ard-2023-224575. doi: 10.1136/ard-2023-224575. Epub ahead of print. PMID: 37495237.

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