Idiopathic Pulmonary Fibrosis, Sarcoidosis
Conditions
Brief summary
The study will measure airway inflammation in probable idiopathic pulmonary fibrosis (IPF) and sarcoidosis as well as in healthy volunteers. This can help understand the molecular basis of these diseases, why these diseases happen, and what makes patients develop lung fibrosis. These insights should one day help to monitor patients and aid in their diagnosis and treatment.
Detailed description
IPF is a progressive disease caused by irreversible scarring of the lung, and disease trajectory is not easily predicted based on clinical measurements. Biomarkers reflective of molecular pathways involved in IPF may help inform patient trajectory, but have been difficult to identify in circulation due to the disease manifesting in the lung. The study team will measure biomarkers from Probable IPF patients, sarcoidosis patients, and healthy volunteers using novel sampling methods involving absorption of upper and lower airway fluids. These novel sampling methods may enable less invasive and potentially more sensitive methods to detect disease activity and will be performed in IPF and sarcoidosis patients during a routine bronchoscopy procedure. The study team will compare the levels of biomarkers that have been shown to be predictive of disease course in airway fluids of probable IPF patients versus sarcoidosis and healthy controls. This study may help understand the molecular basis of IPF, and improve the understanding of diagnosis and treatment.
Interventions
PROCEDUREBronchoscopy for Probable Idiopathic Pulmonary Fibrosis and Sarcoidosis
Blood samples and Nasosorption sampling
Sponsors
Genentech, Inc.
Imperial College London
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
40 Years to 85 Years
Inclusion criteria
* Inclusion Criteria for Probable Idiopathic Pulmonary Fibrosis (IPF) * Adult male or female patients aged 40 to 85 years * Women of childbearing age should not be pregnant, planning to get pregnant or breast-feeding. * Command of the English language to be able to give informed consent. * Probable IPF requiring bronchoscopy to confirm the diagnosis, agreed within the local multi-disciplinary team (MDT).,according to the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/ American Latin Thoracic Association (ATS/ERS/JRS/ALAT) guidelines (2018) (3) * IPF disease diagnosis within the past 5 years * Usual Interstitial Pneumonia (UIP) on HRCT scan. * Recent lung function criteria: * Forced vital capacity (FVC) \>40% of predicted value. * Carbon monoxide diffusing lung capacity (DLco) corrected for haemoglobin \>30% of predicted value Inclusion criteria for Sarcoidosis * Adult male or female patients aged 18 years and over * Women of childbearing age should not be pregnant, planning to get pregnant or breast-feeding. * Clinical symptoms, CT scan and biopsy diagnosis of sarcoidosis * Patients with lung parenchymal disease and pulmonary stage II or more * Recent lung function criteria * FVC\>50% predicted * DLCO \>40% predicted Inclusion criteria for Healthy Volunteers * Age between 40 to 85 years, age and sex to match the group with IPF * Healthy subjects without any diseases that may cause inflammation * Women of childbearing age should not be pregnant, planning to get pregnant or breast-feeding. * Currently non-smokers: see
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Levels of the of biomarker/mediator surfactant protein D (SPD) in bronchial Lining fluid in IPF and sarcoidosis patients | Baseline Bronchoscopy visit | Comparisons will be made of bronchial lining fluid levels of biomarker/mediator surfactant protein D (SPD), in patients with IPF and sarcoidosis. |
| Levels of the biomarker/mediator CCL18 in bronchial Lining fluid in IPF and sarcoidosis patients. | Baseline Bronchoscopy visit | Comparisons will be made of bronchial lining fluid levels of biomarker/mediator CCL18 in patients with IPF and sarcoidosis |
| Levels of the biomarker/mediator CXCL13 in bronchial Lining fluid in IPF and sarcoidosis patients. | Baseline Bronchoscopy visit | Comparisons will be made of bronchial lining fluid levels of biomarker/mediator CXCL13 in patients with IPF and sarcoidosis. |
| Levels of the of biomarker/mediator periostin in bronchial Lining fluid in IPF and sarcoidosis patients. | Baseline Bronchoscopy visit | Comparisons will be made of bronchial lining fluid levels of biomarker/mediator periostin in patients with IPF and sarcoidosis. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Levels of periostin in blood within and across the 3 groups of participants | Through study completion, an average of 1 year | Comparison will be made of periostin levels in blood with nasosorption and bronchosorption levels across the 3 participant groups. |
| Levels of surfactant protein D (SPD in blood within and across the 3 groups of participants | Through study completion, an average of 1 year | Comparison will be made of surfactant protein D (SPD levels in blood with nasosorption and bronchosorption levels across the 3 participant groups |
| Levels of Periostin in nasosorption samples within and across the 3 groups of participants | Through study completion, an average of 1 year | Comparisons will be made of airways levels of biomarker/mediator periostin between patients with IPF and sarcoidosis and health volunteers. |
| Levels of CXCL13 in blood within and across the 3 groups of participants | Through study completion, an average of 1 year | Comparison will be made of CXCL13 levels in blood with nasosorption and bronchosorption levels across the 3 participant groups. |
| Levels of CCL18 in blood within and across the 3 groups of participants | Through study completion, an average of 1 year | Comparison will be made of CCL18 levels in blood with nasosorption and bronchosorption levels across the 3 participant groups. |
| Levels of surfactant protein (SPD) in nasosorption samples within and across the 3 groups | Through study completion, an average of 1 year | Comparisons will be made of airways levels of biomarker/mediator surfactant protein D (SPD) between patients with IPF and sarcoidosis and health volunteers. |
| Levels of CCL18 in nasosorption samples within and across the 3 groups | Through study completion, an average of 1 year | Comparisons will be made of airways levels of biomarker/mediator CCL18 between patients with IPF and sarcoidosis and health volunteers. |
| Levels of CXCL13 in nasosorption samples within and across the 3 groups | Through study completion, an average of 1 year | Comparisons will be made of airways levels of biomarker/mediator CXCL13 between patients with IPF and sarcoidosis and health volunteers. |
Outcome results
None listed