Prospective Evaluation of Biomarker Profiles in Idiopathic Pulmonary Fibrosis

Study Overview
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Brief Summary
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Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal, fibrotic disorder of the lung. The estimated prevalence is 30-80/100,000 in the United States with incidence estimates clearly rising. A major challenge in the care of patients with IPF is determining prognosis. The natural history of IPF is usually one of inexorable decline in lung function, ultimately resulting in death from respiratory failure. However, longitudinal physiologic decline in IPF is heterogeneous and difficult to predict in individual patients. While some patients with IPF may remain stable for years, in others the disease may progress rapidly over a relatively short time. We hypothesize that peripheral blood biomarkers based on extracellular matrix and matrix-modifying molecules will improve prognostication in patients with IPF.

Official Title
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Prospective Evaluation of Biomarker Profiles in Idiopathic Pulmonary Fibrosis

Conditions
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IPF

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age 35-80 years, inclusive Diagnosis of IPF by HRCT or surgical lung biopsy Able to understand and provide informed consent Exclusion Criteria: AE-IPF during the prior year Environmental exposure (occupational, drug, etc.) felt to be the etiology of the interstitial disease. Diagnosis of collagen-vascular conditions according to published American College of Rheumatology criteria. Significant airway obstruction (FEV1/FVC ratio < 0.60) or bronchodilator response, defined as a change in FEV1 ≥ 12% and absolute change > 200 mL OR change in FVC ≥ 12% and absolute change > 200 mL at baseline Partial pressure of arterial oxygen (PaO2) < 55 mm Hg Evidence of active infection Listed for lung transplantation Myocardial infarction, coronary artery bypass, or angioplasty within 6 months Unstable angina pectoris or congestive heart failure requiring hospitalization or deteriorating within 6 months Uncontrolled arrhythmia or hypertension Known HIV, hepatitis C, cirrhosis, or chronic active hepatitis Active substance and/or alcohol abuse If you are pregnant or breastfeeding Any condition other than IPF that is likely to result in your death within the next year Any condition that, in the judgment of the PI, might cause participation in the study to be detrimental to you or that the PI deems makes you a poor candidate

Ages Eligible for Study
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Minimum Age: 35 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with IPF<br>Observation of longitudinal biomarkers in IPF patients | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression-free survival | The primary outcome is your progression free survival as determined by time until any of: death, acute exacerbation of IPF, relative decline in FVC (liters) of at least 10% or DLCO (ml/min/mmHg) of 15% from baseline. | 1 year | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Idiopathic Pulmonary Fibrosis, Biomarkers, Forced Vital Capacity, Diffusion Capacity for Carbon Monoxide, Prognosis