BACKGROUND We have limited data on the treatment of calcinosis cutis associated with systemic sclerosis and dermatomyositis. OBJECTIVE To assess the efficacy and tolerance of available treatments for calcinosis cutis based on previously published studies. METHODS We performed a systematic review of studies published in Medline, Embase, and the Cochrane library during 1980-July 2018. The strength of clinical data was graded according to the modified Oxford Centre for Evidence-Based Medicine levels of evidence. RESULTS In all, 30 studies (288 patients) were included. Eleven therapeutic classes, surgery, and physical treatments were identified as potential treatment options for calcinosis cutis. On the basis of results of a small randomized controlled trial and 4 retrospective studies, low-dose warfarin should not be used for calcinosis cutis (level IB evidence). The results of several studies suggest diltiazem and bisphosphonates might be useful treatment options (level IV). Considering biologic therapies, rituximab has shown promising results in treating both dermatomyositis and systemic sclerosis, whereas tumor necrosis factor inhibitors might be useful for treating juvenile dermatomyositis (level IV). Intralesional sodium thiosulfate might be a promising alternative (level IV). LIMITATIONS Few included studies had a high level of evidence. CONCLUSION This study highlights the efficacy and tolerance profiles of available treatments for calcinosis cutis, with a focus on level of evidence.

中文翻译：

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在系统性硬化症和皮肌炎中治疗角质层角质病：文献综述。

背景技术关于与系统性硬化症和皮肌炎相关的角质层钙化的治疗，我们的数据有限。目的基于先前发表的研究评估可用于皮肤角质层炎的可用疗法的疗效和耐受性。方法我们对1980年7月至2018年7月在Medline，Embase和Cochrane图书馆中发表的研究进行了系统的综述。根据修改后的牛津循证医学中心的证据水平对临床数据的强度进行了分级。结果总共纳入了30项研究（288例患者）。十一种治疗类别，手术和物理疗法被确定为皮肤钙化病的潜在治疗选择。根据一项小型随机对照试验和4项回顾性研究的结果，小剂量华法林不宜用于皮肤角质层炎（IB级证据）。几项研究的结果表明，地尔硫卓和双膦酸盐可能是有用的治疗选择（IV级）。考虑到生物疗法，利妥昔单抗在治疗皮肌炎和系统性硬化症方面均显示出令人鼓舞的结果，而肿瘤坏死因子抑制剂可用于治疗青少年皮肌炎（IV级）。鼻内硫代硫酸钠可能是一种有前途的替代品（IV级）。局限性很少有纳入研究具有高水平的证据。结论本研究重点介绍了可用于皮肤角质病的治疗方法的功效和耐受性，重点是证据水平。考虑到生物疗法，利妥昔单抗在治疗皮肌炎和全身性硬化症方面均显示出令人鼓舞的结果，而肿瘤坏死因子抑制剂可用于治疗青少年皮肌炎（IV级）。鼻内硫代硫酸钠可能是一个有前途的替代品（IV级）。局限性很少有纳入研究具有高水平的证据。结论本研究重点介绍了可用于皮肤角质病的治疗方法的功效和耐受性，重点是证据水平。考虑到生物疗法，利妥昔单抗在治疗皮肌炎和全身性硬化症方面均显示出令人鼓舞的结果，而肿瘤坏死因子抑制剂可用于治疗青少年皮肌炎（IV级）。鼻内硫代硫酸钠可能是一个有前途的替代品（IV级）。局限性很少有纳入研究具有高水平的证据。结论本研究重点介绍了可用于皮肤角质病的治疗方法的功效和耐受性，重点是证据水平。局限性很少有纳入研究具有高水平的证据。结论本研究重点介绍了可用于皮肤角质病的治疗方法的功效和耐受性，重点是证据水平。局限性很少有纳入研究具有高水平的证据。结论本研究重点介绍了可用于皮肤角质病的治疗方法的功效和耐受性，重点是证据水平。