IPF Longitudinal Natural History Study

N/AActive Not RecruitingNCT07560189

Assiut University1,423 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

1,423

Conditions

Idiopathic Pulmonary Fibrosis (IPF)

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

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.

Time frame: 24 months

Secondary Outcomes

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.

Time frame: Up to 24 months

Time to First Respiratory-Related Hospitalization

Time from baseline to first hospitalization related to respiratory deterioration or acute exacerbation.

Time frame: Up to 24 months

All-Cause Mortality

Occurrence of death from any cause during the study follow-up period.

Time frame: 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.

Time frame: 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.

Time frame: Up to 24 months

IPF Longitudinal Natural History Study

Overview

Conditions

Eligibility

Locations (1)

Outcomes

Primary Outcomes

Secondary Outcomes