We updated the design of this site on December 18, 2017. Learn more.
ClinicalTrials.gov
ClinicalTrials.gov Menu

Idiopathic Pulmonary Fibrosis--Pathogenesis and Staging - SCOR in Occupational and Immunological Lung Diseases

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
ClinicalTrials.gov Identifier: NCT00005317
Recruitment Status : Completed
First Posted : May 26, 2000
Last Update Posted : July 12, 2016
Sponsor:
Information provided by:

Study Description
Brief Summary:
To conduct cross-sectional and longitudinal studies of patients with idiopathic pulmonary fibrosis (IPF) and patients with progressive systemic sclerosis (PSS), with and without associated lung disease.

Condition or disease
Lung Diseases Pulmonary Fibrosis Lung Diseases, Interstitial Scleroderma, Systemic

Detailed Description:

BACKGROUND:

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology characterized pathologically by a chronic inflammatory process (alveolitis) that precedes and likely controls the alterations in connective tissue matrix that eventually destroys the normal lung architecture. The mechanisms involved in this process are not known. A complex cell-cell interactive sequence, involving principally neutrophils, lymphocytes, macrophages, fibroblast, and epithelial cells is believed to be responsible.

The SCOR in Occupational and Immunological Lung Diseases was first awarded in December, 1981. The subproject on idiopathic pulmonary fibrosis was first awarded in December, 1986.

DESIGN NARRATIVE:

The cross-sectional comparison examined bronchoalveolar lavage, high resolution, thin-section computer tomography (HRCT), neutrophil or monocyte labeled scintigraphy). The serial, longitudinal evaluation monitored the progression of disease. The study of PSS patients, without disease or with subclinical disease, was particularly useful because it allowed examination of the early events in the pathogenesis of IPF. The long-term goal of the project was to determine what alterations in cellular composition, function and trafficking occurred in the lung parenchyma of patients with IPF and to relate these alterations to the disease stage, prognosis, and therapeutic responsiveness.

The major objectives of the study were: (1) to continue the prospective, longitudinal study of carefully defined cases of IPF; (2) to initiate a study of patients with PSS, a disease that provided a useful paradigm for studying the early events of the disease; (3) to determine the role of the lymphocyte in IPF, by defining the subset of T lymphocytes responsible for modulating macrophage function; (4) to establish the role of non-invasive techniques (bronchoalveolar lavage (BAL), HRCT scanning and scintigraphy -- neutrophil and monocyte labelled cells) in assessing the activity of inflammation in carefully evaluated patients; (5) to determine the relationship of these results to the -- carefully defined and serially obtained -- clinical, radiographical, and physiological findings and to the histopathologic abnormalities (in patients that underwent lung biopsy, predominantly IPF cases). (6) to perform studies utilizing blood, BAL fluid and lung tissue in an effort to understand the pathogenic mechanisms that underlied the inflammatory/immune cellular injury and fibrosis that characterize this disease.

The study completion date listed in this record was obtained from the "Completed Date" entered in the Query View Report System (QVR).


Study Design

Study Type : Observational
Study Start Date : December 1986
Study Completion Date : November 1996


Groups and Cohorts


Outcome Measures

Eligibility Criteria

Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.


Ages Eligible for Study:   up to 100 Years   (Child, Adult, Senior)
Sexes Eligible for Study:   Male
Accepts Healthy Volunteers:   No
Criteria
No eligibility criteria
More Information

Publications:
McCormack FX, King TE Jr, Bucher BL, et al: Surfactant protein A predicts survival in idiopathic pulmonary fibrosis. [published erratum appears in Am J Respir Crit Care Med 1995 Oct;152(4 Pt 1):1425]. Comment in: Am J Respir Crit Care Med 1996 Sep;154(3 Pt 1):825-6. Am J Respir Crit Care Med, 152(2):751-759, 1995.

ClinicalTrials.gov Identifier: NCT00005317     History of Changes
Other Study ID Numbers: 4090
First Posted: May 26, 2000    Key Record Dates
Last Update Posted: July 12, 2016
Last Verified: May 2000

Additional relevant MeSH terms:
Lung Diseases
Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
Lung Diseases, Interstitial
Respiratory Tract Diseases
Idiopathic Interstitial Pneumonias
Connective Tissue Diseases
Skin Diseases
Fibrosis
Scleroderma, Systemic
Scleroderma, Diffuse
Pathologic Processes