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特发性肺纤维化:诊断、生物标志物和新的治疗方案。

Idiopathic pulmonary fibrosis: Diagnosis, biomarkers and newer treatment protocols.

机构信息

NHL MMC, Ahmedabad, India.

B.J. Medical College, Ahmedabad, India.

出版信息

Dis Mon. 2023 Jul;69(7):101484. doi: 10.1016/j.disamonth.2022.101484. Epub 2022 Oct 9.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung condition marked by lung scarring that progresses over time and with usual interstitial pneumonia histology (UIP). It is linked to a worsening cough, dyspnea, and a worse quality of life. Around 3 million persons worldwide suffer from IPF, and the prevalence rises sharply with advancing age. The detection of the UIP pattern, generally using high-resolution CT; lung biopsy may be necessary in certain individuals; the diagnostic approach also includes the elimination of other interstitial lung illnesses or overlapping problems. The UIP pattern is mostly bilateral, peripheral, and basal, with clusters of subpleural cystic airspaces and reticular alterations linked to traction bronchiectasis. Although there are still many uncertainties about how to define susceptibility, it is believed that the molecular mechanisms causing IPF reflect an abnormal reparative response to repeated alveolar epithelial damage in an aging genetically sensitive individual. With the availability of two pharmacotherapeutic drugs, pirfenidone and nintedanib, that slow physiological advancement and potentially increase progression-free survival, significant progress has been made in our knowledge of the clinical treatment of IPF. The goal of current research is to develop early biomarkers for IPF that may include circulating variables, demographic information, and imaging data.

摘要

特发性肺纤维化(IPF)是一种慢性、进行性肺部疾病,其特征是肺部瘢痕随时间推移而进展,并伴有通常的间质性肺炎组织学(UIP)。它与咳嗽加重、呼吸困难和生活质量下降有关。全球约有 300 万人患有 IPF,且患病率随年龄增长急剧上升。UIP 模式的检测通常使用高分辨率 CT;在某些情况下可能需要进行肺活检;诊断方法还包括排除其他间质性肺疾病或重叠问题。UIP 模式主要是双侧、外周和基底的,伴有胸膜下囊状气腔和与牵引性支气管扩张相关的网状改变的簇状。尽管对于如何定义易感性仍存在许多不确定性,但人们认为导致 IPF 的分子机制反映了遗传敏感个体中反复肺泡上皮损伤的异常修复反应。随着两种可减缓生理进展并可能增加无进展生存期的药物治疗药物——吡非尼酮和尼达尼布的出现,我们对 IPF 临床治疗的认识取得了重大进展。目前研究的目标是开发 IPF 的早期生物标志物,这些标志物可能包括循环变量、人口统计学信息和影像学数据。

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