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.

Study Overview

• Status: Active, not recruiting
• Conditions: Idiopathic Pulmonary Fibrosis (IPF)

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 Type: Observational
• Enrollment (Actual): 1423

Contacts and Locations

Study Locations

• Egypt
  • Asyut, Egypt, 71515
    • Faculty Of Medicine - Assiut University Hospitals - Assiut - Egypt

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

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.

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Secondary Outcome Measures

Outcome Measure	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	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

Collaborators and Investigators

• Sponsor: Assiut University
• Investigators: Principal Investigator: Aliae A. Hussien, MD, Assiut University; Principal Investigator: Ahmad M. Shaddad, MD, Assiut University; Principal Investigator: Maiada K. Hashem, MD, Assiut University

Study record dates

Study Major Dates

• Study Start (Actual): April 1, 2022
• Primary Completion (Estimated): August 1, 2026
• Study Completion (Estimated): September 1, 2026

Study Registration Dates

• First Submitted: April 24, 2026
• First Submitted That Met QC Criteria: April 24, 2026
• First Posted (Actual): May 1, 2026

Study Record Updates

• Last Update Posted (Actual): May 1, 2026
• Last Update Submitted That Met QC Criteria: April 24, 2026
• Last Verified: April 1, 2026

More Information

Terms related to this study

• Keywords: mortality; hospitalization; idiopathic pulmonary fibrosis; disease progression; IPF; acute exacerbation; pulmonary fibrosis; natural history; longitudinal cohort
• Additional Relevant MeSH Terms: Pathologic Processes; Disease Attributes; Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial; Fibrosis; Pathological Conditions, Signs and Symptoms; Disease Progression; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: 2025-23-4

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

IPD Plan Description

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.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No