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Management of interstitial lung disease in systemic sclerosis

机译:系统性硬化中间质性肺疾病的治疗

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摘要

Interstitial lung disease (ILD) is very common in systemic sclerosis (SSc) and the leading cause of death. High-resolution computed tomography is a sensitive tool for the diagnosis of ILD in SSc and is abnormal in up to 90% of patients. The most common radiographic and histopathologic pattern seen in these patients is one of nonspecific interstitial pneumonia. Despite the high incidence of disease, the prognosis for most patients is good and those who progress tend to do so early in the course of disease. Treatment options are limited by a paucity of placebo-controlled trials. Data for use of cyclophosphamide and mycophenolate mofetil exist and there is an ongoing trial comparing these two treatments. Although there are limited data to guide us on how to care for these patients, this article proposes a management algorithm. There is ongoing research on new biological treatments as well as the use of biomarkers to predict the course of disease and response to treatment and these may shape the future management of SSc-ILD.
机译:间质性肺疾病(ILD)在系统性硬化症(SSc)中非常常见,并且是主要的死亡原因。高分辨率计算机断层扫描是诊断SSc中ILD的敏感工具,在多达90%的患者中异常。这些患者中最常见的放射学和组织病理学模式是非特异性间质性肺炎之一。尽管疾病高发,但大多数患者的预后良好,那些进展的患者倾向于在疾病早期进行。治疗选择受到安慰剂对照试验的限制。存在使用环磷酰胺和霉酚酸酯的数据,并且正在进行一项比较这两种治疗方法的试验。尽管很少有数据可以指导我们如何护理这些患者,但本文提出了一种管理算法。正在进行有关新的生物治疗方法的研究,以及使用生物标志物预测疾病的进程和对治疗的反应,这些可能会影响SSc-ILD的未来治疗。

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