IPF Longitudinal Natural History Study

Natural History and Temporal Dynamics of Idiopathic Pulmonary Fibrosis: A Longitudinal Cohort Study With Structured Disease Stratification

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.

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.

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