Examination of pirfenidone (Esbriet®) therapy in coal workers' pneumoconiosis (black lung) with pulmonary fibrosis (scarring of the lung).
Examination of pirfenidone (Esbriet®) therapy in coal workers' pneumoconiosis with pulmonary fibrosis associated with radiographic and functional impairment to examine the reduction in progression of disease, reduction of exacerbation rates and possible reduction of inflammatory biomarkers as an index of clinical response.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
50
see previous entries
Pulmonary Research of Abingdon, LLC
Abingdon, Virginia, United States
Forced Vital Capacity (FVC)
Primary objective for this study is to determine the rate of decline ( relative change in FVC (% and ml) in patients with coal workers' pneumoconiosis (Black Lung) with pulmonary fibrosis (CWP/PMF) during treatment with pirfenidone (Esbriet®).
Time frame: 12 months minimum
Forced Expiratory Volume in 1 second (FEV1)
rate of decline (relative change in % and ml )of FEV1 compared to baseline assessments established at enrollment.
Time frame: 12 months minimum
Diffusing capacity of the lung for carbon monoxide (DLCO)
rate of decline (relative change in %) of DLCO compared to baseline assessments established at enrollment.
Time frame: 12 months minimum
6 minute walk test
rate of decline of 6 minute walk test compared to baseline assessments established at enrollment.
Time frame: 12 months minimum
Chest CT
Chest CT scans will be reviewed to determine if radiographic progression occurs during therapy.
Time frame: 12 months
Inflammatory biomarkers
The reduction of inflammatory biomarkers (Interleukin-6 \[IL-6\], Transforming Growth Factor Beta 1 \[TGF-β 1\] and Tumor Necrosis Factor Alpha \[TNF-α\]) and the potential reduction correlation with change in lung function
Time frame: 12 months minimum
St. George's Respiratory Questionnaire (SGRQ)
Change in total score compared to baseline. Total score summarizes the impact of the disease on overall health status. Scores range from 0 - 100, with higher scores indicating more limitations.
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Time frame: 12 months minimum