Systemic Sclerosis Associated Interstitial Lung Disease and Nintedanib: A Rare Disease and a Promising Drug.
Systemic Sclerosis Associated Interstitial Lung Disease and Nintedanib: A Rare Disease and a Promising Drug.
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DOI：
10.7759/cureus.16404
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发表时间：
2021-07
期刊：
影响因子：
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通讯作者：
Cancarevic I
中科院分区：
文献类型：
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作者：
Kafle S;Thapa Magar M;Patel P;Poudel A;Cancarevic I
系统性硬化症相关间质性肺病（SSc-ILD）是一种罕见的进行性疾病，最终导致肺纤维化。Nintedanib是一种酪氨酸激酶抑制剂，是一种被广泛接受的治疗特发性肺纤维化（IPF）的药物，IPF是一种与SSc-ILD在病理过程上有一些相似之处的疾病。在这篇综述中，我们的目的是讨论SSc-ILD的发病机制和尼达尼布在SSc-ILD治疗中的总体作用。SSc-ILD涉及多种病理介质，参与最终导致肺纤维化的各种途径。SSc-ILD的发病机制是一个复杂的现象，仍需进一步研究。尼达尼布通过减少病理过程的进展证明了其治疗SSc-ILD的有效性。它在SSc-ILD治疗中的临床意义也得到了证实。然而，目前可用的文献没有任何证据来比较尼达尼布与现有的治疗方式，如环磷酰胺（CYC）、霉酚酸酯（MMF）和硫唑嘌呤（AZT）的有效性。目前的文献也缺乏关于尼达尼布对SSc-ILD患者的长期影响的信息。因此，为了创建更好的循证治疗指南，我们建议研究人员进行随机临床试验，将尼达尼布与MMF、CYC、AZT等进行比较，并继续监测尼达尼布的长期后果。
Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a rare disease with a progressive nature, eventually leading to lung fibrosis. Nintedanib, a tyrosine kinase inhibitor, is a widely accepted drug for treating idiopathic pulmonary fibrosis (IPF), a disease that shares some similarities with SSc-ILD regarding pathological disease processes. In this review, we aim to discuss the pathogenesis of SSc-ILD and the overall role of nintedanib in the management of SSc-ILD. SSc-ILD involves multiple pathological mediators contributing to various pathways that ultimately cause lung fibrosis. The pathogenesis of SSc-ILD is a complex phenomenon and still needs further study. Nintedanib has demonstrated its efficacy in the treatment of SSc-ILD by reducing the progression of the pathological process. It has also proven its clinical significance in the management of SSc-ILD. However, the currently available literature does not have any evidence to compare the effectiveness of nintedanib with the already available treatment modalities such as cyclophosphamide (CYC), mycophenolate mofetil (MMF), and azathioprine (AZT). The current literature also lacks information about nintedanib's long-term consequences on patients with SSc-ILD. Therefore, to create better evidence-based treatment guidelines, we recommend that researchers conduct randomized clinical trials comparing nintedanib to MMF, CYC, AZT, etc., and continue surveillance to explore the long-term consequences of nintedanib.
影响因子：
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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
通讯作者：
Suarez-Cuartin, Guillermo