Prospective Evaluation of Biomarker Profiles in Idiopathic Pulmonary Fibrosis

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.

Study Overview

• Status: Completed
• Conditions: IPF

• Study Type: Observational
• Enrollment (Actual): 43

Contacts and Locations

Study Locations

• United States
  • Michigan
    • Ann Arbor, Michigan, United States, 48109
      • University of Michigan Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 35 years to 80 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

The study population includes subjects with IPF identified via the University of Michigan Interstitial Lung Disease Clinical-Radiologic-Pathologic Conference. The investigators will only enroll subjects in whom an IPF diagnosis is firmly established.

Description

Inclusion Criteria:

1. Age 35-80 years, inclusive
2. Diagnosis of IPF by HRCT or surgical lung biopsy
3. Able to understand and provide informed consent

Exclusion Criteria:

1. AE-IPF during the prior year
2. Environmental exposure (occupational, drug, etc.) felt to be the etiology of the interstitial disease.
3. Diagnosis of collagen-vascular conditions according to published American College of Rheumatology criteria.
4. 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
5. Partial pressure of arterial oxygen (PaO2) < 55 mm Hg
6. Evidence of active infection
7. Listed for lung transplantation
8. Myocardial infarction, coronary artery bypass, or angioplasty within 6 months
9. Unstable angina pectoris or congestive heart failure requiring hospitalization or deteriorating within 6 months
10. Uncontrolled arrhythmia or hypertension
11. Known HIV, hepatitis C, cirrhosis, or chronic active hepatitis
12. Active substance and/or alcohol abuse
13. If you are pregnant or breastfeeding
14. Any condition other than IPF that is likely to result in your death within the next year
15. 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

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Patients with IPF; Observation of longitudinal biomarkers in IPF patients

What is the study measuring?

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

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Longitudinal change in biomarker levels	In exploratory analyses, longitudinal change in your biomarker expression will be correlated with your disease progression to determine if change in biomarker levels over time predict subsequent disease progression.	1 year

Collaborators and Investigators

• Sponsor: University of Michigan
• Collaborators: National Heart, Lung, and Blood Institute (NHLBI); Brown University
• Investigators: Principal Investigator: Eric S White, MD, University of Michigan

Study record dates

Study Major Dates

• Study Start: August 1, 2013
• Primary Completion (Actual): July 1, 2017
• Study Completion (Actual): July 1, 2017

Study Registration Dates

• First Submitted: May 28, 2014
• First Submitted That Met QC Criteria: May 29, 2014
• First Posted (Estimate): May 30, 2014

Study Record Updates

• Last Update Posted (Actual): July 26, 2017
• Last Update Submitted That Met QC Criteria: July 25, 2017
• Last Verified: July 1, 2017

More Information

Terms related to this study

• Keywords: Prognosis; Biomarkers; Idiopathic Pulmonary Fibrosis; Forced Vital Capacity; Diffusion Capacity for Carbon Monoxide
• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Fibrosis; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: UM HUM00004076; R01HL109118 (U.S. NIH Grant/Contract)