A Prognostic Transcriptomic Signature for Chronic Hypersensitivity Pneumonitis

Study Overview
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Brief Summary
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Up to 135 patients with hypersensitivity pneumonitis will be enrolled at 7 clinical centers across the United States. Patients will be followed for 24 months to determine if biomarkers in the blood can predict disease progression.

Detailed Description
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Hypersensitivity pneumonitis is an immunologically mediated form of lung disease, resulting from inhalation exposure to a large variety of antigens. For unknown reasons, a subgroup of patients with HP without a known inciting antigen exposure develops chronic disease with progressive pulmonary fibrosis (the leading cause of death). Accurately identifying patients at risk of disease progression is necessary for prognosis and therapy. Enrolled subjects will be followed for 24 months and complete 5 visits to one of the study centers. Blood biomarkers will be collected in addition to other clinical data to determine if these biomarkers can predict disease progression.

Official Title
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Development and Validation of a Prognostic Transcriptomic Signature for Chronic Hypersensitivity Pneumonitis

Conditions
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Hypersensitivity Pneumonitis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of chronic hypersensitivity pneumonitis Age 18 through 85 years. Diagnosis of chronic hypersensitivity pneumonitis by HRCT Able to understand and sign a written informed consent form. Able to understand the importance of adherence to the study protocol and willing to follow all study requirements Exclusion Criteria: Not a suitable candidate for enrollment or unlikely to comply with the requirements of this study Known explanation for the interstitial lung disease Clinical diagnosis of any connective tissue disease Listed or expected to receive a lung transplant within 4 months from enrollment Pregnant women

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 85 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Time to absolute FVC (percent predicted) decline ≥10 percent |  | Up to 24 months | 
| Time to absolute DLCO (percent predicted) decline ≥10 percent |  | Up to 24 months | 
| Time to relative FVC (percent predicted) decline ≥10 percent |  | Up to 24 months | 
| Time to relative DLCO (percent predicted) decline ≥10 percent |  | Up to 24 months | 
| Time to relative DLCO (percent predicted) decline ≥15 percent |  | Up to 24 months | 
| Time to first adjudicated acute chronic hypersensitivity pneumonitis exacerbation |  | Up to 24 months | 
| Need for a new course of oral or intravenous steroids |  | Up to 24 months | 
| Time to death from any cause |  | Up to 24 months | 
| Change from baseline in St. George's Respiratory Questionnaire total score at month 6 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 6 months | 
| Change from baseline in St. George's Respiratory Questionnaire total score at month 12 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 12 months | 
| Change from baseline in St. George's Respiratory Questionnaire total score at month 18 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 18 months | 
| Change from baseline in St. George's Respiratory Questionnaire total score at month 24 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 24 months | 
| Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 6 | Scores range from 0 to 120, with higher scores indicating greater breathlessness. | 6 months | 
| Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 12 | Scores range from 0 to 120, with higher scores indicating greater breathlessness. | 12 months | 
| Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 18 | Scores range from 0 to 120, with higher scores indicating greater breathlessness. | 18 months | 
| Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 24 | Scores range from 0 to 120, with higher scores indicating greater breathlessness. | 24 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 6 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 6 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 12 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 12 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 18 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 18 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 24 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 24 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 6 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 6 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 12 |  | 12 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 18 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 18 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 24 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 24 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 6 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 6 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 12 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 12 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 18 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 18 months | 
| Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 24 | The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life. | 24 months | 
| Change from baseline in CT visual (≥10 percent) score at month 12 | Visual scores range from 0 to 100, with higher scores indicating more lung fibrosis. | 12 months | 
| Change from baseline in CT visual (≥10 percent) score at month 24 | Visual scores range from 0 to 100, with higher scores indicating more lung fibrosis. | 24 months | 
| Change from baseline in CT quantitative (≥3.4) score at month 12 | CT quantitative scores range from 0 to no upper limits, with higher scores indicating more lung fibrosis. | 12 months | 
| Change from baseline in CT quantitative (≥3.4) score at month 24 | CT quantitative scores range from 0 to no upper limits, with higher scores indicating more lung fibrosis. | 24 months | 
| Change from baseline in 6-min walk distance at month 6 |  | 6 months | 
| Change from baseline in 6-min walk distance at month 12 |  | 12 months | 
| Change from baseline in 6-min walk distance at month 18 |  | 18 months | 
| Change from baseline in 6-min walk distance at month 24 |  | 24 months | 
| Rate of decline in FVC (percent predicted) over 24 months |  | Up to 24 months | 
| Rate of decline in FVC (ml) over 24 months |  | Up to 24 months | 
| Rate of decline in DLCO (percent predicted) over 24 months |  | Up to 24 months | 
| Rate of decline in DLCO (mmol/min/kpa) over 24 months |  | Up to 24 months |