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特发性肺纤维化的早期诊断与治疗:一篇叙述性综述

Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review.

作者信息

Alsomali Hana, Palmer Evelyn, Aujayeb Avinash, Funston Wendy

机构信息

Faculty of Medical Sciences, Newcastle University, Newcastle Upon Tyne, NE1 7RU, UK.

Department of Respiratory Medicine, The Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, NE1 4LP, UK.

出版信息

Pulm Ther. 2023 Jun;9(2):177-193. doi: 10.1007/s41030-023-00216-0. Epub 2023 Feb 11.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3-5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

摘要

特发性肺纤维化(IPF)是一种病因不明的慢性、进行性纤维化间质性肺疾病。患者通常在数月至数年的时间里出现慢性呼吸困难和咳嗽症状。IPF预后较差,如果不治疗,从诊断起平均预期寿命为3至5年。两种抗纤维化药物(尼达尼布和吡非尼酮)已被批准用于治疗IPF。这些药物通过减少肺功能下降来减缓疾病进展。早期诊断对于确保及时选择治疗方法和改善预后至关重要。高分辨率计算机断层扫描(HRCT)在IPF的诊断中起主要作用。在这篇叙述性综述中,我们讨论了早期诊断的重要性、初级保健医生的认识、肺癌筛查计划与IPF早期检测,以及获取抗纤维化药物的障碍。

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