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ACR/EULAR classification criteria for CPPD disease

ACR/EULAR classification criteria for CPPD disease
Definition of criteria
The CPPD disease classification criteria should be applied in the following order:
  1. Entry criterion: Ever had at least 1 episode of joint pain, swelling, or tenderness.*
  2. Absolute exclusion criteria: All symptoms are more likely explained by an alternative condition (such as rheumatoid arthritis, gout, psoriatic arthritis, OA, etc).
  3. Sufficient criteria: Presence of either crowned dens syndrome or synovial fluid analysis demonstrating CPP crystals in a joint with swelling, tenderness, or pain.

An individual is classified as having CPPD disease if the entry criterion is met, exclusion criteria are not met, and at least 1 sufficient criterion is fulfilled.

If none of the sufficient criteria are present, an individual is classified as having CPPD disease if the sum of the criteria is >56 points.
Scoring of criteria
Items can be scored if they were ever present during a patient's lifetime. If a patient fulfills >1 item in a given domain, only the highest weighted item will be scored. Imaging of at least 1 symptomatic joint by CR, US, CT, or DECT is required.
Domains and levels Points
  1. Age at onset of joint symptoms (pain, swelling, and/or tenderness)
≤60 years 0
>60 years 4
  1. Time course and symptoms of inflammatory arthritisΔ
No persistent or typical inflammatory arthritis 0
Persistent inflammatory arthritis 9
One typical acute arthritis episode 12
More than 1 typical acute arthritis episode 16
  1. Sites of typical episode(s) of inflammatory arthritis in peripheral joints
First MTP joint –6
No typical episode(s) 0
Joint(s) other than wrist, knee, or first MTP joint 5
Wrist 8
Knee 9
  1. Related metabolic diseases
None 0
Present 6
  1. Synovial fluid crystal analysis from a symptomatic joint§
CPP crystals absent on ≥2 occasions –7
CPP crystals absent on 1 occasion –1
Not performed 0
  1. OA of hand/wrist on imaging (defined as present if the K/L score is ≥2)
None of the below findings or no wrist/hand imaging performed 0
OA of radiocarpal joints bilaterally 2
≥2 of the following findings: STT joint OA without first CMC joint OA; second MCP joint OA; third MCP joint OA 7
  1. Imaging evidence of CPPD in symptomatic peripheral joint(s)¥
None on US, CT, or DECT (and absent on CR or CR not performed) –4
None on CR (and US, CT, DECT not performed) 0
Present on either CR, US, CT, or DECT 16
  1. Number of peripheral joints with evidence of CPPD on any imaging modality regardless of symptoms¥
None 0
1 16
2 to 3 23
≥4 25

CMC: carpometacarpal; CR: conventional radiography; K/L: Kellgren/Lawrence; MCP: metacarpophalangeal; MTP: metatarsophalangeal; STT: scaphotrapeziotrapezoid; US: ultrasound.

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

¶ 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 localized 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 computed tomography (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, ie, 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).

Δ 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.

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

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

¥ Imaging of at least 1 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.
From: Abhishek A, Tedeschi SK, Pascart T, et al. The 2023 ACR/EULAR Classification Criteria for calcium pyrophosphate deposition disease. Arthritis Rheumatol 2023; 75:1703. Copyright © 2023 The Authors. Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
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