Experience With Nintedanib in Severe Pulmonary Fibrosis Associated With Systemic Sclerosis: A Case Series.
Experience With Nintedanib in Severe Pulmonary Fibrosis Associated With Systemic Sclerosis: A Case Series.
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DOI：
10.1016/j.opresp.2020.100080
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发表时间：
2021-01
影响因子：
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通讯作者：
Suarez-Cuartin, Guillermo
中科院分区：
文献类型：
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作者：
Bordas-Martinez, Jaume;Llanos-Gonzalez, Ana Belen;Jodar-Masanes, Ramon;Vicens-Zygmunt, Vanesa;Bermudo, Guadalupe;Luburich, Patricio;Dorca, Jordi;Molina-Molina, Maria;Suarez-Cuartin, Guillermo
系统性硬化症是一种自身免疫性结缔组织疾病，通过炎症、血管损伤和肺纤维化影响多个器官。1在过去的世纪，SSc患者的主要死亡原因是肾危象，但由于治疗的进步，这一事件的发生率从42%下降到6%。2最近的数据显示，相关的间质性肺疾病（ILD）和肺动脉高压是最常见的SSC相关死亡原因，
Systemic sclerosis (SSc) is an autoimmune connective tissue disease, which affects several organs through inflammation, vascular injury and lung fibrosis. 1 In the past century, the primary cause of death among SSc patients had been renal crisis, but the rate of this event decreased from 42% to 6% due to advances in treatment. 2 Recent data show that associated interstitial lung disease (ILD) and pulmonary hypertension are the most frequent SSc-related cause of death, with a reported mortality rate of 33–35% for