Calcinosis in systemic sclerosis.

Calcinosis in systemic sclerosis.
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DOI:
10.1097/bor.0000000000000896
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发表时间:
2022-11-01
影响因子:
5.1
通讯作者:
--
中科院分区:
医学2区
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--
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提供与系统性硬化症(SSc)相关的皮肤钙质沉着症的患病率、发病机制、诊断和治疗的最新信息。观察性研究表明,钙质沉着症的患病率存在种族和地理差异。除了临床和血清学相关性之外,生物化学研究和体内模型还试图解释其发病机制背后的理论,包括长期炎症状态,机械应力,缺氧以及骨和磷酸盐代谢失调。长期使用质子泵抑制剂可能会增加SSc患者发生钙质沉着的风险。很少有单中心观察性研究显示米诺环素和局部硫代硫酸钠有轻度获益。皮肤钙质沉着症是不溶性钙在皮肤和皮下组织中的沉积。它影响高达40%的SSc患者,并导致显著的发病率。长期病程、血管功能障碍和骨质疏松与钙质沉着有关。无机焦磷酸盐和成纤维细胞生长因子-23水平的改变与磷酸盐代谢失调有关,磷酸盐代谢失调可能导致SSc中的钙质沉着。X线平片可帮助诊断和定量钙质沉着。在可行的情况下,手术治疗仍然是最有效的治疗方法。目前,没有药物治疗在大型随机对照试验中证明有效。
To provide updated information on the prevalence, pathogenesis, diagnostics, and therapeutics of calcinosis cutis associated with systemic sclerosis (SSc). Observational studies show ethnic and geographical differences in the prevalence of calcinosis. In addition to clinical and serological associations, biochemical studies and in vivo models have attempted to explain theories behind its pathogenesis including prolonged state of inflammation, mechanical stress, hypoxia and dysregulation in bone and phosphate metabolism. Long-term use of proton pump inhibitors may increase the risk for calcinosis in SSc. Few single center observational studies have shown mild benefit with minocycline and topical sodium thiosulfate. Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It affects up to 40% of SSc patients and causes significant morbidity. Long disease duration, features of vascular dysfunction, and osteoporosis have been associated with calcinosis. Altered levels of inorganic pyrophosphate and fibroblast growth factor-23 have been implicated in dysregulated phosphate metabolism that may lead to calcinosis in SSc. Plain radiography can help with diagnosis and quantifying the calcinosis burden. Surgical treatment remains the most effective therapy when feasible. At present, no medical therapies have proven efficacy in large randomized controlled trials.
患有多发性外根吸收的系统性硬化症患者钙质沉着的松质骨样组织替代物。
DOI: 10.1016/j.bonr.2021.101165
发表时间: 2022-06
期刊: Bone reports
影响因子: 2.5
作者:
Memida T;Matsuda S;Nakamoto T;Ouhara K;Kajiya M;Hirata S;Sugiyama E;Kakimoto N;Mizuno N
通讯作者: Mizuno N