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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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. |
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| Measure | Description | Time Frame |
|---|---|---|
| Time to First Composite Clinical Event (Acute Exacerbation, Hospitalization, or Death) in 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. | 24 months |
| Measure | Description | Time Frame |
|---|---|---|
| Time to First Acute Exacerbation of Idiopathic Pulmonary Fibrosis | Time from baseline enrollment to the first occurrence of acute exacerbation of idiopathic pulmonary fibrosis during follow-up. | Up to 24 months |
| Time to First Respiratory-Related Hospitalization |
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Inclusion Criteria:
Exclusion Criteria:
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Adult patients diagnosed with idiopathic pulmonary fibrosis enrolled in a longitudinal observational cohort at Assiut University between 2022 and 2026. Participants undergo standardized baseline evaluation and structured follow-up to document disease progression, acute exacerbations, hospitalization, and mortality. The cohort represents real-world clinical practice and includes patients eligible for longitudinal assessment without intervention assignment.
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| Name | Affiliation | Role |
|---|---|---|
| Ahmad M. shaddad, MD | Assiut University | Principal Investigator |
| Aliae A. Hussien, MD | Assiut University | Principal Investigator |
| Maiada K. Hashem, MD | Assiut University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Faculty Of Medicine - Assiut University Hospitals - Assiut - Egypt | Asyut | 71515 | Egypt |
Individual participant data underlying the results reported in this study will not be publicly shared. Data are governed by the Assiut University IPF Research Program policies and institutional regulations. De-identified data may be considered for sharing upon reasonable request to the corresponding investigator, subject to institutional review, ethical approval, and execution of a formal data use agreement.
Analytical code and model specifications are not publicly distributed. Access may be considered under controlled conditions in accordance with institutional policy, intellectual property considerations, and approved collaborative agreements.
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| ID | Term |
|---|---|
| D054990 | Idiopathic Pulmonary Fibrosis |
| D018450 | Disease Progression |
| D011658 | Pulmonary Fibrosis |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D020969 | Disease Attributes |
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Time from baseline to first hospitalization related to respiratory deterioration or acute exacerbation. |
| Up to 24 months |
| All-Cause Mortality | Occurrence of death from any cause during the study follow-up period. | Up to 24 months |
| Inflammatory Burnout Index (IBI)-Defined Disease Activity | Evaluation of disease activity using the Inflammatory Burnout Index (IBI), a predefined analytical framework for dynamic disease characterization (Zenodo DOI: 10.5281/zenodo.18843181). Applied observationally without influencing clinical management. | Up to 12 months |
| Severity-Phenotype-Dynamics (SPD) Integrated Disease States | Assessment of integrated disease states using the Severity-Phenotype-Dynamics (SPD) framework combining severity, phenotype, and dynamic disease behavior (Zenodo DOI: 10.5281/zenodo.18843362). Applied for observational stratification only. | Up to 24 months |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D005355 | Fibrosis |