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当代简明综述 2023:间质性肺疾病。

Contemporary Concise Review 2023: Interstitial lung disease.

机构信息

School of Medicine, University of Western Australia, Crawley, Western Australia, Australia.

Institute for Respiratory Health, Nedlands, Western Australia, Australia.

出版信息

Respirology. 2024 Dec;29(12):1095-1100. doi: 10.1111/resp.14848. Epub 2024 Oct 22.

Abstract

In this review, we have discussed several important developments in 2023 in Interstitial Lung Disease (ILD). The association of pollution with genetic predispositions increased the risk of Idiopathic Pulmonary Fibrosis (IPF). An interesting comorbidity of malnutrition was not adequately recognized in ILD. Novel genes have been identified in IPF involving predominantly short telomere length and surfactant protein production leading to alveolar epithelial cell dysfunction. Genetics also predicted progression in IPF. Crosstalk between vascular endothelial cells and fibroblasts in IPF mediated by bone morphogenic protein signalling may be important for remodelling of the lung. A novel modality for monitoring of disease included the 4-min gait speed. New treatment modalities include inhaled pirfenidone, efzofitimod, for sarcoidosis, and earlier use of immunosuppression in connective tissue disease-ILD.

摘要

在这篇综述中,我们讨论了 2023 年间间质性肺病(ILD)领域的几个重要进展。污染与遗传易感性的关联增加了特发性肺纤维化(IPF)的风险。ILD 中营养不良的一种有趣的合并症并未得到充分认识。在 IPF 中发现了涉及短端粒长度和表面活性剂蛋白产生的新基因,导致肺泡上皮细胞功能障碍。遗传学也预测了 IPF 的进展。IPF 中血管内皮细胞和成纤维细胞之间通过骨形态发生蛋白信号的串扰可能对肺重塑很重要。一种监测疾病的新方法包括 4 分钟步行速度。新的治疗方法包括吸入吡非尼酮、特立氟胺治疗结节病,以及更早地在结缔组织病-ILD 中使用免疫抑制。

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