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A Prognostic Transcriptomic Signature for Chronic Hypersensitivity Pneumonitis

Active, not recruiting
Conditions
Hypersensitivity Pneumonitis
Registration Number
NCT04844359
Lead Sponsor
National Jewish Health
Brief Summary

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.

Detailed Description

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.

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
137
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

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Time to absolute FVC (percent predicted) decline ≥10 percentUp to 24 months
Time to relative DLCO (percent predicted) decline ≥15 percentUp to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 66 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 2424 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 66 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 66 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 1212 months

The total score ranges from 0 to 100, with higher scores indicating worse health-related quality of life.

Time to absolute DLCO (percent predicted) decline ≥10 percentUp to 24 months
Time to relative DLCO (percent predicted) decline ≥10 percentUp to 24 months
Time to first adjudicated acute chronic hypersensitivity pneumonitis exacerbationUp to 24 months
Change from baseline in dyspnea using St. George's Respiratory Questionnaire impact score at month 66 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 1212 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 2424 months

Visual scores range from 0 to 100, with higher scores indicating more lung fibrosis.

Rate of decline in DLCO (percent predicted) over 24 monthsUp to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 1212 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 percentUp to 24 months
Need for a new course of oral or intravenous steroidsUp to 24 months
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 1212 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 1818 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 2424 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 2424 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 1212 months
Rate of decline in FVC (percent predicted) over 24 monthsUp to 24 months
Time to death from any causeUp to 24 months
Change from baseline in St. George's Respiratory Questionnaire total score at month 1818 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 2424 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 66 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 1212 months
Change from baseline in CT visual (≥10 percent) score at month 1212 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 1212 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 monthsUp to 24 months
Change from baseline in dyspnea using University of California San Diego Shortness of Breath Questionnaire at month 1818 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 1818 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 66 months
Change from baseline in 6-min walk distance at month 1818 months
Change from baseline in dyspnea using St. George's Respiratory Questionnaire activity score at month 1818 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 2424 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 2424 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 2424 months
Rate of decline in DLCO (mmol/min/kpa) over 24 monthsUp to 24 months
Secondary Outcome Measures
NameTimeMethod

Trial Locations

Locations (7)

University of Arizona

🇺🇸

Tucson, Arizona, United States

University of Chicago

🇺🇸

Chicago, Illinois, United States

Mayo Clinic

🇺🇸

Rochester, Minnesota, United States

University of California Davis

🇺🇸

Sacramento, California, United States

National Jewish Health

🇺🇸

Denver, Colorado, United States

University of Texas Southwestern

🇺🇸

Dallas, Texas, United States

University of Utah

🇺🇸

Salt Lake City, Utah, United States

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