Pulmonary Fibrosis
Conditions
Keywords
"Lung[A04.400]"
Brief summary
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
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.
Interventions
None listed
Sponsors
University of Pittsburgh
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Genetic Polymorphisms in Idiopathic Pulmonary Fibrosis (IPF) | Sample collection will occur up to 5 years based on the current rate of sample collection. | Blood samples will be collected to validate in a large cohort the per- allele associations of prespecified SNPs with IPF case status adjusted for age , sex. smoking, and principal components of ancestry. |
Countries
United States
Contacts
CONTACTMichelle F MacPherson, MAT
CONTACTMichelle Meyers, BSN
PRINCIPAL_INVESTIGATORKevin F Gibson, MD
University of Pittsburgh, Simmons Center for ILD
Outcome results
None listed