Natural History of Bronchiectasis
- Conditions
- Cystic FibrosisCommon Variable ImmunodeficiencyAutoimmune DiseaseBronchiectasis
- Registration Number
- NCT00943514
- Brief Summary
Background:
* Bronchiectasis is a disease characterized by airways that are inflamed, abnormally dilated, and chronically infected. Individuals with bronchiectasis have a history of chronic and recurring respiratory infections. Depending on the underlying cause, these infections may involve the entire respiratory tract, resulting in sinus, ear, and lung disease.
* Bronchiectasis continues to be a significant problem in developing countries and in specific groups of individuals, particularly in people who have cystic fibrosis. Although treatments are available or under development for bronchiectasis related to cystic fibrosis, many of the disease-specific treatments may not be effective for bronchiectasis not related to cystic fibrosis.
Objectives:
- To study the natural history of bronchiectasis to identify inherited and immune factors that may explain why certain individuals have chronic recurring infections.
Eligibility:
* Individuals 5 years of age and older who have an established diagnosis of bronchiectasis or a history of chronic/recurring respiratory infections.
* Direct family members (e.g., parents, siblings, children) of patients in the study may also be asked to participate.
Design:
* Potential participants will be screened with an initial clinic evaluation and full medical history, as well as a general quality of life and respiratory symptom questionnaire.
* The following standard procedures may be done as part of the study: air sampling from the nose; imaging studies, which may include an x-ray or computed tomography (CT), lung function tests; and collection of samples of blood, urine, and sputum (phlegm or mucus). Other tests may be performed as required by the researchers, and will be explained to patients as needed.
* Both patients and relatives (if asked to participate) will provide the following samples: blood or buccal (cheek swab) cells for genetic testing, sputum, and urine.
* To prevent infections and potential disease progression, patients may receive standard medical care and treatment for bronchiectasis and related infections during this protocol.
- Detailed Description
Bronchiectasis, or abnormal dilation of the airways, is a condition typically characterized by chronic and recurring respiratory tract infections. Frequently, depending on the underlying cause, these infections involve the entire respiratory tract resulting in sinus, ear, and lung disease. This condition used to be more common in children prior to immunizations for childhood infections. It continues to be a significant problem in developing countries and in specific groups of individuals in the U.S. Cystic fibrosis (CF) is the most commonly associated genetic condition and tremendous strides have been made in recent years in understanding the mechanisms of this disease that are leading to a multitude of emerging novel treatment strategies. The mechanisms of other causes for bronchiectasis have not evolved to this degree, and many of the disease-specific treatments being assessed for cystic fibrosis may not be effective for non-CF bronchiectasis. Often bronchiectasis can be associated with chronic infections from environmental germs such as the nontuberculous mycobacteria.
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- All
- Target Recruitment
- 900
Not provided
Not provided
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method Identify causes and/or underlying conditions associated with bronchiectasis Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. Identify causes and/or underlying conditions associated with bronchiectasis.
- Secondary Outcome Measures
Name Time Method Monitor a cohort of bronchiectasis patients to better understand factors associated with progression of disease and to assess outcomes of management and therapeutic strategies. Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. 1)Assessment of genetic, systemic immune, and/or epithelial surface defense mechanisms: a) Collection and storage of blood and sputum b) Use and storage of other relevant biologic specimens 2) Assessment: a) St. George s Respiratory Questionnaire b) Pulmonary Symptom Severity Score c) Medical Research Council Dyspnea Scale d) Six-minute walk e) Computerized tomography score f) Spirometry: g) Lower airway microbiology: Gram s stain/bacterial cultures, modified AFB smear/Nocardia cultures, wet mount/fungal cultures, and AFBsmears/mycobacterial cultures as well as novel biomarkers ofmycobacteria,antimicrobial resistance and microbial changes on theprogression of bronchiectasis. h) Inflammatory markers: sedimentation rate, C-reactive protein, beta-2 microglobulin i) Screen adult patients for anxiety and depression and correlate with standardized measures of respiratory symptoms, disease severity, and quality of life.
Define mechanisms and pathophysiology for the development and progression of bronchiectasis Patients will be seen at least every 6 months for the first 2 years, and then at 1-5 year intervals to follow the course of their disease for 5 years or until they no longer wish to return. Genetic, systemic immune, and/or epithelial surface defense mechanisms involved in airway infection susceptibility and/or development of bronchiectasis through the
Trial Locations
- Locations (1)
National Institutes of Health Clinical Center
🇺🇸Bethesda, Maryland, United States
National Institutes of Health Clinical Center🇺🇸Bethesda, Maryland, United StatesFor more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR)Contact800-411-1222ccopr@nih.gov