Prospective Observation of Fibrosis in the Lung Clinical Endpoints Study (PROFILE)
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Idiopathic pulmonary fibrosis (IPF) is a progressive scarring condition of the lungs the cause of which is unknown.There are currently no effective treatments for IPF and the condition tends to cause progressive disability and death with an average survival of 3.5 years from diagnosis. The condition is responsible for the deaths of 4000 people per year in the UK. At present the definite diagnosis of IPF rests on the identification of a specific pattern of fibrosis when a section of fibrotic lung tissue is examined under a microscope. Unfortunately, the process of obtaining a lung biopsy requires an operation and is not with out risk. The investigators hope to identify specific markers in the blood and lungs of patients with IPF that will enable the condition to be diagnosed without biopsy. Furthermore, the investigators hope to identify indicators(biomarkers) that will predict which patients have more aggressive and progressive disease and also to identify biomarkers that might be useful in identifying a response to treatment and might therefore be used in future clinical trials in IPF. As well as looking at markers in the blood and lungs the investigators also plan to assess the use of daily home lung function measurement and a computerised technique for analyzing lung sounds to see if these are investigations that are able to predict the development of worsening lung fibrosis.
Discover and validate novel biomarkers and gene expression profiles for use in subsequent clinical studies in patients with idiopathic pulmonary fibrosis.
Secondary Outcome Measures
Study disease behaviour [ Time Frame: 3 years ]
Prospectively evaluate longitudinal disease behavior in patients with IPF and other fibrotic lung diseases of unknown cause with a view to developing composite clinical endpoints for subsequent use in clinical studies in patients with pulmonary fibrosis.
Differentiate IPF from NSIP [ Time Frame: 3 years ]
Identify differences in the pathogenetic mechanisms involved in the development of different types of fibrosis in patients with fibrotic lung disease of unknown cause.
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Ages Eligible for Study:
18 Years and older (Adult, Senior)
Sexes Eligible for Study:
Accepts Healthy Volunteers:
Subjects will be recruited from patients refered to the Interstitial Lung Disease Unit of the Royal Brompton Hospital.
Individuals over the age of 18 with a diagnosis of definite or probable IPF or definite or probable fibrotic NSIP as defined by the ATS/ERS consensus classification
Patients with co-existent conditions known to be associated with the development of fibrotic lung disease will be excluded.
connective tissue disease
suspected drug-induced lung disease
asbestosis or other asbestos related disease (pleural plaques, mesothelioma, asbestos pleural effusions)
granulomatous disease including sarcoidosis.
Patients with an auto-immune profile considered diagnostic for a specific connective tissue disease will be excluded, even in the absence of systemic symptoms.
Non-specific rises in auto antibodies e.g. rheumatoid factor, anti-nuclear antibody etc. will not be used to exclude individuals from the study.
Patients with co-morbid disease that in the opinion of the investigators gives them an expected life expectancy of less than one year will be excluded from the study.
Patients involved in clinical trials assessing novel IPF therapies will be excluded from enrolment in this study.