The Edinburgh Lung Fibrosis Molecular Endotyping (ELFMEN) Study
试验速览
- 阶段
- 不适用
- 状态
- Enrolling By Invitation
- 入组人数
- 800
- 主要终点
- Time to death (all cause)
研究概览
简要总结
To prospectively study novel blood and lung biomarkers of disease activity in patients with IPF and other interstitial lung disease with the aims of prognostic modelling and disease clustering
详细描述
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is a progressive form of lung scarring for which there is no proven treatment. Steroids and other potentially toxic drugs are often used but their efficacy is uncertain. The management of patients with IPF is particularly complex because firstly there are a number of closely related fibrosing lung conditions that 'look' like IPF but in which the prognosis is generally better and which more often respond to steroids and secondly even within the group of patients with IPF, there is a variability in the rate of progression so that it is hard to provide individual patients with a reasonable estimate of prognosis.
Currently the best the investigators can offer patients with IPF or other fibrotic interstitial lung disease is serial measurement of lung function over time and observation of decline. The uncertainty regarding disease prognosis and progression in a given patient is hugely unsettling for both the individual and the clinician. A far more powerful tool would be a measurement or 'biomarker' of disease activity that one could monitor from the time of diagnosis and throughout the illness and which predicted for decline in lung function or poor prognosis. This biomarker (or biomarkers) might include molecules associated with inflammation and scarring in blood or in lung fluid, new more sensitive measures of lung function (e.g. the six-minute walking test) or novel non-invasive imaging methods. Once established, a robust biomarker would serve several important functions including:
- Prognostication of individuals with IPF and other fibrotic ILDs
- Identifying targets for potential new therapies in IPF and other fibtroic ILDs
- A marker of disease response to drugs used in therapeutic trials in IPF and other fibrotic ILDs.
- A means of distinguishing definite IPF from other closely related conditions;
AIMS To prospectively study novel blood and lung biomarkers of disease activity in patients with IPF and other interstitial lung disease
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 99 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •patients with interstitial lung disease attending the Edinburgh Lung Fibrosis service
排除标准
- •candidates not a suitable for enrolment or unlikely to comply with the requirements of this study, in the opinion of the investigator, will be excluded.
结局指标
主要结局
Time to death (all cause)
时间窗: 15 years
Biomarkers that are associated with increased all-cause mortality
次要结局
- Rate of decline in vital capacity(15 years)
- Time to death (lung-related)(15 years)
- Rate of decline in TLCO(15 years)