Natural History and Temporal Dynamics of Idiopathic Pulmonary Fibrosis: A Longitudinal Cohort Study With Structured Disease Stratification
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 入组人数
- 1,423
- 试验地点
- 1
- 主要终点
- Time to First Composite Clinical Event (Acute Exacerbation, Hospitalization, or Death) in 24 months
研究概览
简要总结
This is a longitudinal observational cohort study of patients with idiopathic pulmonary fibrosis (IPF) conducted within the Assiut University IPF Research Program between 2022 and 2026. The study aims to characterize the natural history, temporal disease dynamics, event patterns, and clinical outcomes of IPF over a two-year follow-up period.
Primary analyses focus on disease progression, acute exacerbation, hospitalization, mortality, and final clinical state. Secondary analyses include prespecified evaluation of the Inflammatory Burnout Index (IBI; Zenodo DOI: 10.5281/zenodo.18843181) and the Severity-Phenotype-Dynamics integration framework (SPD; Zenodo DOI: 10.5281/zenodo.18843362) as observational analytical frameworks for disease activity and multidimensional disease characterization. These frameworks are applied for research stratification only and do not influence treatment allocation, clinical management, or follow-up decisions.
详细描述
This observational cohort study follows patients with idiopathic pulmonary fibrosis using predefined longitudinal clinical, physiological, radiological, and outcome assessments. The study is designed to describe disease burden, temporal progression, event distribution, and end-state outcomes in a real-world IPF cohort.
Eligible participants are adults with a clinical and radiological diagnosis of IPF according to standard diagnostic assessment, with available baseline evaluation and planned longitudinal follow-up. Patients are followed for up to two years to document acute exacerbations, respiratory hospitalization, functional decline, mortality, and final clinical status.
The study includes secondary analytical evaluation of two predefined disease-characterization frameworks. The Inflammatory Burnout Index (IBI) is used to evaluate dynamic inflammatory-fibrotic balance and functional reserve. The SPD framework integrates disease severity, phenotype, and dynamic disease behavior into a multidimensional observational stratification structure. Both frameworks are archived with Zenodo DOIs and are evaluated without modifying patient care.
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
- •Adults aged ≥18 years.
- •Diagnosis of idiopathic pulmonary fibrosis based on clinical and radiological assessment according to standard guidelines.
- •Availability of baseline clinical, functional, and radiological evaluation.
- •Enrollment within the Assiut University IPF Research Program.
- •Ability to undergo longitudinal follow-up and outcome assessment.
排除标准
- •Interstitial lung disease other than idiopathic pulmonary fibrosis
- •Alternative diagnosis inconsistent with IPF based on clinical or radiological evaluation
- •Incomplete baseline data preventing cohort classification
- •Refusal to participate or inability to complete follow-up
- •End-stage disease at baseline requiring invasive mechanical ventilation or terminal care
- •Active malignancy or severe comorbidity expected to dominate short-term prognosis
- •Long-term systemic immunosuppressive therapy not aligned with standard IPF management
研究组 & 干预措施
Idiopathic Pulmonary Fibrosis Cohort
This cohort includes adult patients with idiopathic pulmonary fibrosis enrolled in a longitudinal observational study conducted between 2022 and 2026 at Assiut University. Participants undergo predefined follow-up assessments to document clinical outcomes, including disease progression, acute exacerbations, hospitalization, and mortality. No interventions are assigned, and all data are collected as part of routine clinical care and structured observational follow-up.
结局指标
主要结局
Time to First Composite Clinical Event (Acute Exacerbation, Hospitalization, or Death) in 24 months
时间窗: 24 months
Time from cohort entry (baseline enrollment) to the first occurrence of any of the following events: acute exacerbation of idiopathic pulmonary fibrosis, respiratory-related hospitalization, or all-cause mortality. Events are prospectively recorded during longitudinal follow-up within the study period.
次要结局
- Time to First Acute Exacerbation of Idiopathic Pulmonary Fibrosis(Up to 24 months)
- Time to First Respiratory-Related Hospitalization(Up to 24 months)
- All-Cause Mortality(Up to 24 months)
- Inflammatory Burnout Index (IBI)-Defined Disease Activity(Up to 12 months)
- Severity-Phenotype-Dynamics (SPD) Integrated Disease States(Up to 24 months)
研究者
Ahmad Shaddad
Associate Professor
Assiut University