Natural History and Temporal Dynamics of Idiopathic Pulmonary Fibrosis: A Longitudinal Cohort Study With Structured Disease Stratification
Overview
- Phase
- Not Applicable
- Status
- Active, not recruiting
- Sponsor
- Assiut University
- Enrollment
- 1,423
- Locations
- 1
- Primary Endpoint
- Time to First Composite Clinical Event (Acute Exacerbation, Hospitalization, or Death) in 24 months
Overview
Brief Summary
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.
Detailed Description
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.
Study Design
- Study Type
- Observational
- Observational Model
- Cohort
- Time Perspective
- Prospective
Eligibility Criteria
- Ages
- 18 Years to — (Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- No
Inclusion Criteria
- •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.
Exclusion Criteria
- •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
Arms & Interventions
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.
Outcomes
Primary Outcomes
Time to First Composite Clinical Event (Acute Exacerbation, Hospitalization, or Death) in 24 months
Time Frame: 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.
Secondary Outcomes
- 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)
Investigators
Ahmad Shaddad
Associate Professor
Assiut University