Genetic Polymorphisms in Idiopathic Pulmonary Fibrosis (IPF)

NCT ID: NCT00258570

Last Updated: 2025-11-12

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

2000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2003-01-31

Study Completion Date

2035-07-31

Brief Summary

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The purposes of this study are:

* to determine if there are specific genetic traits that might explain why patients have developed pulmonary fibrosis;
* to determine if specific genetic traits account for differing patterns of inflammation and scar tissue that has formed in the patient's lungs.

Detailed Description

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Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology that is characterized by the insidious development of lung fibrosis ultimately leading to distortion of the lung architecture, respiratory failure, and death. IPF is one of several entities associated with pulmonary fibrosis called the idiopathic interstitial pneumonias (IIP). Based on the histopathologic features of the fibrotic process, it is possible to identify four distinct entities: usual interstitial pneumonia (UIP) (synonymous with IPF), nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia DIP), and acute interstitial pneumonia (AIP) (Hamman-Rich lung). Each type appears to have different clinical progression and a different response to anti-inflammatory therapy. Our overall objective is to elucidate the molecular pathogenesis of IPF (UIP) by identifying factors that determine host susceptibility to this disease. We hypothesize that patients who develop pulmonary fibrosis, have a genetic propensity to abnormal lung repair that leads to fibrosis after acute lung injury. We further hypothesize that these genetic susceptibilities may determine if the pathologic process in the lung after an insult becomes UIP, AIP, NSIP, or DIP. To explore these hypotheses we propose to characterize the genetic polymorphisms in candidate genes involved in inflammation, matrix turnover, fibroblast proliferation and differentiation, and epithelial cell proliferation; and to correlate this with indices of disease progression.

Conditions

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Pulmonary Fibrosis

Keywords

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"Lung[A04.400]"

Study Design

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Observational Model Type

COHORT

Study Time Perspective

OTHER

Eligibility Criteria

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Inclusion Criteria

* 18 years of age or older
* Diagnosis of pulmonary fibrosis confirmed by physical examination, pulmonary function testing, chest X-ray, and computed tomography (CT) scans.
* Adult patients who are seeking treatment at the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease.

Exclusion Criteria

* Under 18 years of age
* Non-fibrotic ILD
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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University of Pittsburgh

OTHER

Sponsor Role lead

Responsible Party

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Kevin F. Gibson

Professor of Medicine

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Kevin F Gibson, MD

Role: PRINCIPAL_INVESTIGATOR

University of Pittsburgh, Simmons Center for ILD

Locations

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University of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status RECRUITING

Countries

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United States

Central Contacts

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Michelle F MacPherson, MAT

Role: CONTACT

Phone: 412-647-4537

Email: [email protected]

Michelle Meyers, BSN

Role: CONTACT

Phone: 412-692-2149

Email: [email protected]

Facility Contacts

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Michelle F MacPherson, MAT

Role: primary

Michelle Meyers, BSN

Role: backup

Related Links

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http://www.upmc.com/services/pulmonology/interstitial-lung-disease/

The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease is designed to provide patient-friendly advice and support for people with idiopathic pulmonary fibrosis.

Other Identifiers

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STUDY20030223

Identifier Type: -

Identifier Source: org_study_id