Calcium pyrophosphate deposition disease

IF 15 1区 医学 Q1 RHEUMATOLOGY Lancet Rheumatology Pub Date : 2024-07-29 DOI:10.1016/S2665-9913(24)00122-X
Prof Tristan Pascart MD , Georgios Filippou MD , Prof Frédéric Lioté MD , Silvia Sirotti MD , Charlotte Jauffret MD , Prof Abhishek Abhishek PhD
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Abstract

Calcium pyrophosphate deposition (CPPD) disease is a consequence of the immune response to the pathological presence of calcium pyrophosphate (CPP) crystals inside joints, which causes acute or chronic inflammatory arthritis. CPPD is strongly associated with cartilage degradation and osteoarthritis, although the direction of causality is unclear. This clinical presentation is called CPPD with osteoarthritis. Although direct evidence is scarce, CPPD disease might be the most common cause of inflammatory arthritis in older people (aged >60 years). CPPD is caused by elevated extracellular-pyrophosphate concentrations in the cartilage and causes inflammation by activation of the NLRP3 inflammasome. Common risk factors for CPPD disease include ageing and previous joint injury. It is uncommonly associated with metabolic conditions (eg, hyperparathyroidism, haemochromatosis, hypomagnesaemia, and hypophosphatasia) and genetic variants (eg, in the ANKH and osteoprotegerin genes). Apart from the detection of CPP crystals in synovial fluid, imaging evidence of CPPD in joints by mainly conventional radiography, and increasingly ultrasonography, has a central role in the diagnosis of CPPD disease. CT is useful in showing calcification in axial joints such as in patients with crowned dens syndrome. To date, no treatment is effective in dissolving CPP crystals, which explains why control of inflammation is currently the main focus of therapeutic strategies. Prednisone might provide the best benefit–risk ratio for the treatment of acute CPP-crystal arthritis, but low-dose colchicine is also effective with a risk of mild diarrhoea. Limited evidence suggests that colchicine, low-dose weekly methotrexate, and hydroxychloroquine might be effective in the prophylaxis of recurrent flares and in the management of persistent CPP-crystal inflammatory arthritis. Additionally, biologics inhibiting IL-1 and IL-6 might have a role in the management of refractory disease.
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焦磷酸钙沉积症。
焦磷酸钙沉积症(CPPD)是关节内焦磷酸钙(CPP)结晶的病理存在引起免疫反应的结果,会导致急性或慢性炎症性关节炎。CPPD 与软骨退化和骨关节炎密切相关,但因果关系尚不明确。这种临床表现被称为 CPPD 伴骨关节炎。虽然缺乏直接证据,但 CPPD 疾病可能是老年人(年龄大于 60 岁)最常见的炎症性关节炎病因。CPPD是由软骨中细胞外焦磷酸浓度升高引起的,并通过激活NLRP3炎性体导致炎症。CPPD疾病的常见风险因素包括年龄增长和既往关节损伤。与代谢性疾病(如甲状旁腺功能亢进、血色素沉着病、低镁血症和低磷血症)和基因变异(如 ANKH 和骨保护蛋白基因)有关的情况并不常见。除了检测滑液中的 CPP 晶体外,主要通过常规放射学检查和越来越多的超声波检查获得关节内 CPPD 的影像学证据在 CPPD 疾病的诊断中起着核心作用。CT 对于显示轴关节的钙化非常有用,例如冠状沟综合征患者。迄今为止,还没有一种治疗方法能有效溶解 CPP 晶体,这也解释了为什么控制炎症是目前治疗策略的重点。泼尼松可能是治疗急性 CPP 晶体关节炎的最佳效益-风险比,但小剂量秋水仙碱也很有效,但有轻度腹泻的风险。有限的证据表明,秋水仙碱、每周一次的小剂量甲氨蝶呤和羟氯喹可能对预防复发和治疗持续性 CPP 晶体炎性关节炎有效。此外,抑制IL-1和IL-6的生物制剂也可用于治疗难治性疾病。
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来源期刊
Lancet Rheumatology
Lancet Rheumatology RHEUMATOLOGY-
CiteScore
34.70
自引率
3.10%
发文量
279
期刊介绍: The Lancet Rheumatology, an independent journal, is dedicated to publishing content relevant to rheumatology specialists worldwide. It focuses on studies that advance clinical practice, challenge existing norms, and advocate for changes in health policy. The journal covers clinical research, particularly clinical trials, expert reviews, and thought-provoking commentary on the diagnosis, classification, management, and prevention of rheumatic diseases, including arthritis, musculoskeletal disorders, connective tissue diseases, and immune system disorders. Additionally, it publishes high-quality translational studies supported by robust clinical data, prioritizing those that identify potential new therapeutic targets, advance precision medicine efforts, or directly contribute to future clinical trials. With its strong clinical orientation, The Lancet Rheumatology serves as an independent voice for the rheumatology community, advocating strongly for the enhancement of patients' lives affected by rheumatic diseases worldwide.
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