Department of Rheumatology, Kings College Hospital, Denmark Hill, London, UK.
Curr Opin Rheumatol. 2012 Mar;24(2):158-64. doi: 10.1097/BOR.0b013e32834ff5cd.
Calcinosis is a recognized manifestation of many connective-tissue diseases, especially juvenile dermatomyositis (JDM) and systemic sclerosis; however, little is known about the pathogenesis and the treatment of this condition. The purpose of this review is to discuss the most recently published data about calcinosis in connective-tissue diseases with emphasis on the pathogenesis and its treatment.
Calcinosis is more common in patients with sustained disease-activity and longer disease duration. JDM patients with anti-p140 antibodies and tumour necrosis factor (TNF) α-308AA allele are at an increased risk. Low levels of the calcium-regulating proteins, fetuin-A and osteopontin, have been found in the serum of patients with JDM. Macrophages and cytokines interleukin (IL) 6, IL-1β and TNFα isolated from the calcific tissues in JDM have also been implicated in the pathologic process. Raised tissue expression of advanced glycation end products and their receptor has been noted in patients with systemic sclerosis and systemic lupus erythematosus with calcinosis.
Many agents have been used for treatment of calcinosis but none has been accepted as a standard therapy. Case studies have shown that aggressive treatment of the underling inflammatory condition with intravenous immunoglobulin, anti TNF agents, thalidomide and haematopoietic stem cell transplantation has also led to improvement of the calcinosis. Aggressive management of the underlying inflammatory condition should help in treating as well as decreasing the incidence of calcinosis. Some case studies have focused on agents such as warfarin, bisphosphonates, diltiazem and others, which are primarily aimed at treating the process of calcinosis with varying success.
钙沉积是许多结缔组织疾病的公认表现，尤其是幼年特发性皮肌炎（JDM）和系统性硬化症；然而，对于这种疾病的发病机制和治疗方法知之甚少。本综述的目的是讨论最近发表的关于结缔组织疾病中钙沉积的文献，重点讨论其发病机制和治疗方法。
钙沉积在疾病活动持续时间长和疾病持续时间长的患者中更为常见。具有抗 p140 抗体和肿瘤坏死因子（TNF）α-308AA 等位基因的 JDM 患者发病风险增加。在 JDM 患者的血清中发现了钙调节蛋白胎球蛋白-A 和骨桥蛋白的水平较低。从 JDM 钙化组织中分离出的巨噬细胞和细胞因子白细胞介素（IL）6、IL-1β和 TNFα也被认为参与了病理过程。在有钙沉积的系统性硬化症和系统性红斑狼疮患者中，组织中高级糖基化终产物及其受体的表达升高。
许多药物已被用于治疗钙沉积，但没有一种被接受为标准治疗。病例研究表明，用静脉注射免疫球蛋白、抗 TNF 药物、沙利度胺和造血干细胞移植积极治疗潜在的炎症状况，也可改善钙沉积。积极管理潜在的炎症状况有助于治疗和减少钙沉积的发生。一些病例研究集中在华法林、双膦酸盐、地尔硫卓等药物上，这些药物主要针对钙沉积过程，取得了不同程度的成功。
