Molecular Diagnosis of Idiopathic Interstitial Pneumonias: a Prospective Study
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 55
- 试验地点
- 1
- 主要终点
- Reproducibility of gene expression profiles on surgical lung biopsies
研究概览
简要总结
Molecular diagnosis of idiopathic interstitial pneumonias is an innovative way to potentially improve the diagnostic accuracy of surgical lung biopsies (SLBs), introducing molecular classifiers of idiopathic pulmonary fibrosis (IPF) vs. non-specific interstitial pneumonia (NSIP) vs. chronic hypersensitivity pneumonitis (CHP).
The investigators hypothesize that pre-defined gene expression profiles previously identified on large lung explants can still be identified and reproducible on smaller, clinically available surgical lung biopsies (SLBs), and can be used to increase diagnostic accuracy during multi-disciplinary discussion.
The investigators also hypothesize that the expression level of individual, preselected genes that accurately differentiate IPF from NSIP and CHP on lung explants can be reproduced on SLBs.
The investigators will isolate RNA from SLBs obtained from patients with IIP and perform microarray analysis to verify the reproducibility of gene expression profiles on SLBs. Individual genes expression levels will be determined by RT-PCR.
The diagnosis will be determined by MDD and further validated by prospective follow-up of patients for a period of 3 years.
The investigators will assess the impact of molecular diagnostic techniques on interobserver agreement during multi-disciplinary discussion.
The investigators will prospectively follow the clinical course of patients after SLB for a period of 3 years to validate the diagnosis, and asses the diagnostic accuracy of molecular techniques.
详细描述
Aim of the study This is a prospective, observational trial to collect samples of SLB, extract RNA, analyze transcriptional profiles by microarray, and validate previously identified gene expression profiles and individual genes expression levels against the diagnosis of IIP (IPF vs. NSIP vs. CHP), defined by multi-disciplinary discussion, and further confirmed by subsequent clinical course. There is no experimental drug involved.
Specific objectives:
- Test and validate the reproducibility of gene expression profiles of IPF and NSIP on the transcriptome obtained from surgical lung biopsies (lung homogenates), prospectively collected.
- Test the gene expression profiles of IPF, NSIP and CHP, respectively, as predictors of 3-year survival from the time of biopsy.
- Test individual gene expression levels (markers of NSIP or IPF) in lung homogenates from SLB with real-time RT-PCR, as discriminators of IPF vs. NSIP.
- Test and validate molecular predictors of lung-transplant survival regardless of the underlying interstitial pattern.
Although the diagnosis of IPF or NSIP will be established by multi-disciplinary discussion, as per guidelines, in order to further validate gene signatures and singles genes as reliable discriminators of IPF vs. NSIP, we will prospectively follow the clinical course of patients for a period of 3 years, to make sure that the clinical course is consistent with either IPF (worse) or NSIP (better). The average clinical courses of IPF and NSIP are clearly established by our 6-year interstitial lung disease database experience.
Assessment of expected outcomes:
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 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- 未提供
排除标准
- 未提供
结局指标
主要结局
Reproducibility of gene expression profiles on surgical lung biopsies
时间窗: February 2019-January 2022
Proportion of patients (% of the total) with reproducible predefined gene expression profiles of either IPF or NSIP on surgical lung biopsy.
次要结局
- Prognostic impact of selected genes expression levels(February 2019-January 2025)
- Prognostic impact of gene expression profiles of IPF and NSIP(February 2019-January 2025)