Development and Validation of a Prognostic Transcriptomic Signature for Chronic Hypersensitivity Pneumonitis
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 入组人数
- 137
- 试验地点
- 9
- 主要终点
- Time to absolute DLCO (percent predicted) decline ≥10 percent
研究概览
简要总结
Up to 150 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.
详细描述
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 85 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •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
结局指标
主要结局
Time to absolute DLCO (percent predicted) decline ≥10 percent
时间窗: Up to 24 months
Time to relative DLCO (percent predicted) decline ≥10 percent
时间窗: Up to 24 months
Time to first adjudicated acute chronic hypersensitivity pneumonitis exacerbation
时间窗: Up to 24 months
Time to relative DLCO (percent predicted) decline ≥15 percent
时间窗: Up to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 6
时间窗: 6 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in St. George's Respiratory Questionnaire total score at month 24
时间窗: 24 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 6
时间窗: 6 months
Scores range from 0 to 120, with higher scores indicating greater breathlessness.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 6
时间窗: 6 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 12
时间窗: 12 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Time to absolute FVC (percent predicted) decline ≥10 percent
时间窗: Up to 24 months
Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 6
时间窗: 6 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 12
时间窗: 12 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in CT visual (≥10 percent) score at month 24
时间窗: 24 months
Visual scores range from 0 to 100, with higher scores indicating more lung fibrosis.
Rate of decline in DLCO (percent predicted) over 24 months
时间窗: Up to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 12
时间窗: 12 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Time to relative FVC (percent predicted) decline ≥10 percent
时间窗: Up to 24 months
Need for a new course of oral or intravenous steroids
时间窗: Up to 24 months
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 12
时间窗: 12 months
Scores range from 0 to 120, with higher scores indicating greater breathlessness.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 18
时间窗: 18 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire symptoms score at month 24
时间窗: 24 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in CT quantitative (≥3.4) score at month 24
时间窗: 24 months
CT quantitative scores range from 0 to no upper limits, with higher scores indicating more lung fibrosis.
Change from baseline in 6-min walk distance at month 12
时间窗: 12 months
Rate of decline in FVC (percent predicted) over 24 months
时间窗: Up to 24 months
Time to death from any cause
时间窗: Up to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 18
时间窗: 18 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 24
时间窗: 24 months
Scores range from 0 to 120, with higher scores indicating greater breathlessness.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 6
时间窗: 6 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 12
时间窗: 12 months
Change from baseline in CT visual (≥10 percent) score at month 12
时间窗: 12 months
Visual scores range from 0 to 100, with higher scores indicating more lung fibrosis.
Change from baseline in CT quantitative (≥3.4) score at month 12
时间窗: 12 months
CT quantitative scores range from 0 to no upper limits, with higher scores indicating more lung fibrosis.
Rate of decline in FVC (ml) over 24 months
时间窗: Up to 24 months
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 18
时间窗: 18 months
Scores range from 0 to 120, with higher scores indicating greater breathlessness.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 18
时间窗: 18 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in 6-min walk distance at month 6
时间窗: 6 months
Change from baseline in 6-min walk distance at month 18
时间窗: 18 months
Rate of decline in DLCO (mmol/min/kpa) over 24 months
时间窗: Up to 24 months
Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 18
时间窗: 18 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 24
时间窗: 24 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 24
时间窗: 24 months
The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.
Change from baseline in 6-min walk distance at month 24
时间窗: 24 months
次要结局
未报告次要终点
研究者
Evans Fernandez Perez
Associate Professor
National Jewish Health