Potential Role of JAK Inhibitors in the Treatment of Systemic Sclerosis-Associated Interstitial Lung Disease: A Narrative Review from Pathogenesis to Real-Life Data.
Potential Role of JAK Inhibitors in the Treatment of Systemic Sclerosis-Associated Interstitial Lung Disease: A Narrative Review from Pathogenesis to Real-Life Data.
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DOI：
10.3390/life12122101
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发表时间：
2022-12-14
期刊：
影响因子：
3.2
通讯作者：
Guiducci, Serena
中科院分区：
文献类型：
--
作者：
Fiorentini, Elisa;Bonomi, Francesco;Peretti, Silvia;Orlandi, Martina;Lepri, Gemma;Matucci Cerinic, Marco;Bellando Randone, Silvia;Guiducci, Serena
背景资料：系统性硬化症相关间质性肺病（SSc-ILD）是SSc最相关的并发症之一，也是死亡的主要原因。SSc-ILD的发病机制涉及多种细胞类型和不同分子途径的复杂相互作用，炎症和纤维化均为病理学标志。到目前为止，还没有能够针对疾病的两个组成部分的治疗方法。Janus激酶抑制剂（JAKinib）代表了一种有趣的治疗选择，因为它们发挥抗炎和抗纤维化特性。研究方法：在此，我们对JAKinib在SSc-ILD中的潜在作用进行了叙述性综述，以定义最新技术水平并评价此类治疗背后的发病机理。结果：目前，很少有研究调查SSc-ILD对JAKinib治疗的反应。分析了来自三项临床研究和四份病例报告的数据，93.5%接受JAKinib治疗的患者的SSc-ILD进展不明显。结论：JAKinibs治疗SSc-ILD的有效性证据很少，但很有希望。JAKinibs可能是SSc-ILD的一种令人感兴趣的治疗方法，因为它们可能抑制纤维化过程并具有抗炎作用。此外，在一些研究中，JAKinibs还显示对肺动脉高压（PAH）有潜在影响，这是SSc的另一种威胁性并发症。需要更多数据来确定JAKinib在SSc-ILD治疗中的作用。
Background: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is one of the most relevant complications of SSc and the major cause of death. The pathogenesis of SSc-ILD involves a complex interplay of multiple cell types and different molecular pathways, with both inflammation and fibrosis as pathological hallmarks. To date, there are no treatments able to target both components of the disease. Janus kinase inhibitors (JAKinibs) represent an interesting therapeutic option because they exert both anti-inflammatory and anti-fibrotic properties. Methods: Here, we performed a narrative review concerning the potential role of JAKinibs in SSc-ILD to define the state of art and to evaluate the pathogenetic rationale behind this type of treatment. Results: Currently, few studies investigated SSc-ILD response to JAKinibs treatment. Data were analyzed from three clinical studies and four case reports and progression of SSc-ILD was not evident in 93.5% of patients treated with JAKinibs. Conclusions: Available evidence of efficacy of JAKinibs in SSc-ILD is sparse but promising. JAKinibs could be an interesting treatment in SSc-ILD because of their potential inhibition of the fibrotic processes combined with their anti-inflammatory action. Moreover, JAKinibs were also shown in some studies to have a potential effect on pulmonary arterial hypertension (PAH), another threatening complication in SSc. More data are necessary to define JAKinibs role in SSc-ILD treatment.
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影响因子：
5.8
作者：
Milara J;Hernandez G;Ballester B;Morell A;Roger I;Montero P;Escrivá J;Lloris JM;Molina-Molina M;Morcillo E;Cortijo J
通讯作者：
Cortijo J
影响因子：
4
作者：
Karalilova RV;Batalov ZA;Sapundzhieva TL;Matucci-Cerinic M;Batalov AZ
通讯作者：
Batalov AZ
影响因子：
9.6
作者：
Chaisson, Neal F.;Hassoun, Paul M.
通讯作者：
Hassoun, Paul M.
影响因子：
5.6
作者：
Febvre-James, Marie;Lecureur, Valerie;Fardel, Olivier
通讯作者：
Fardel, Olivier
DOI：
10.1164/rccm.2106012
发表时间：
2002-06-15
影响因子：
24.7
作者：
Bouros, D;Wells, AU;du Bois, RM
通讯作者：
du Bois, RM