Academic Rheumatology, University of Nottingham, Nottingham, UK
Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
Ann Rheum Dis. 2023 Oct;82(10):1248-1257. doi: 10.1136/ard-2023-224575. Epub 2023 Jul 26.
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.
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.
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).
The 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field.
焦磷酸钙沉积（CPPD）病较为常见且表现多样，但目前尚无针对此类症状性关节炎的验证分类标准。美国风湿病学会（ACR）和欧洲抗风湿病联盟（EULAR）制定了首个针对症状性 CPPD 病的验证分类标准。
在 ACR 和 EULAR 的支持下，一个由多国研究人员组成的团队采用既定方法制定了这些疾病分类标准。该团队列出了候选项目清单，并对其定义进行了细化，收集了未经身份识别的患者概况，评估了候选项目与 CPPD 病之间关联的强度，制定了分类标准框架，并使用多标准决策分析来定义标准权重和分类阈值评分。该标准在独立队列中得到了验证。
在存在关节疼痛、肿胀或压痛（纳入标准）且这些症状不能完全用其他疾病解释的患者（排除标准）中，存在冠状突综合征或滑液中存在焦磷酸钙晶体足以将患者归类为 CPPD 病。如果没有这些发现，则可以使用加权标准（包括临床特征、相关代谢紊乱以及实验室和影像学检查结果），总分>56 分，来对 CPPD 病进行分类。在原始队列（190 例 CPPD 病例，148 例类似病例）中，这些标准的敏感性为 92.2%，特异性为 87.9%，而在验证队列（251 例 CPPD 病例，162 例类似病例）中，敏感性为 99.2%，特异性为 92.5%。
2023 年 ACR/EULAR CPPD 病分类标准具有出色的性能特征，将有助于该领域的研究。
