BronchiectasisNCT00943514
Natural History of Bronchiectasis
An observational study of Bronchiectasis and Cystic Fibrosis, run by National Heart, Lung, and Blood Institute (NHLBI) at 1 site in United States.
Trial summary
Bronchiectasis is a chronic inflammatory and muco-obstructive condition that results in abnormal dilation of the airways, decline in lung function, frequent exacerbations, and chronic respiratory tract infections. Cystic fibrosis (CF) is the most well studied genetic condition that causes bronchiectasis. Read more about this study
- Who is it for?
- Ages 5 to 100 yearsHealthy volunteers can take part
- Conditions the study is for
- Bronchiectasis, Cystic Fibrosis, Autoimmune Disease
- How many people
- 900 participants
- Where
- 1 siteUnited States
- Study dates
- 1 Aug 2009to not given
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 5 to 100 years
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
Study population: Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections and/or bronchiectasis will be eligible for participation. Relatives including 5 years and above.
Who may be able to join
- Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections and/or bronchiectasis will be eligible for participation.
- Males and females will be considered without regard to race or ethnicity or upper age limit.
- Emphasis will be primarily on non CF bronchiectasis for elucidation of mechanisms bronchiectasis development and infection susceptibility. However, select patients with cystic fibrosis or acquired immune defects (such as HIV) may be studied if relevant host defects are suspected, if needed for comparison purposes, or to evaluate and study the management of chronic pathogen associated conditions such as nontuberculous mycobacterial infections or allergic bronchopulmonary aspergillosis.
- Subjects must have a primary or referring physician
- Subjects must be willing to have samples (blood, tissue, sputum, and stool) stored
- Inclusion Criteria for Relatives including children ages 5 years and above; relatives without a known diagnosis of bronchiectasis, including those who are asymptomatic, may be enrolled if they are willing to complete study assessments. These participants will be used for comparative analyses and/or screening for previously undiagnosed disease.
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- Rationale for inclusion of children:
- The research that is conducted on this protocol is regulated by Health and Human Services, and in compliance with federal guidelines Title 45 Code of Federal Regulations, Part 46, Subpart D. The groups of children that may be included are:
- Affected minors: Bronchiectasis may have early onset and a significant impact on the lives of these patients. It is important to include this population in our study in order to understand the natural history and progression of this condition in children as they often have physiological differences as compared to adult patients. Affected minors may undergo testing that will be tailored to their specific clinical presentation.
- Minors that are related to affected individuals: Children that are relatives of index patients may not be affected or show symptoms. It is important for relatives including minor relatives to be enrolled in our study, in order to help us identify the possible conditions like pre-bronchiectasis to better understand the mechanism of the disease progression. Unaffected child relatives may only undergo tests that are no greater than minimal risk.
- Rational for inclusion of affected pregnant and or nursing women: Pregnant and or nursing women including pregnant minors may be included in accordance with Federal Regulations at Subpart B of 45 CFR 46. Bronchiectasis-related symptoms and respiratory status may change during pregnancy. Including pregnant participants in this observational natural history protocol will allow the study to capture longitudinal changes in clinical status and other relevant data during pregnancy and the postpartum period.
- Study procedures conducted during pregnancy and breastfeeding will be limited to those determined to pose no more than minimal risk to the participant and fetus or breastfeeding infant. Procedures considered greater than minimal risk -including computed tomography scans, bronchoscopy with bronchoalveolar lavage and endobronchial biopsy, and research blood collection- will not be performed during pregnancy or breastfeeding. These procedures may be completed after the participant is no longer pregnant and, when applicable, has discontinued breastfeeding, in accordance with the protocol and investigator judgment.
Who cannot join
- <!-- -->
- Patients who are unable or unwilling to provide informed consent
- Any patient who, in the opinion of the Investigator, is unable or unwilling to comply with regular follow-up or is unlikely to provide pertinent information regarding disease progression or response to treatment may be excluded from longitudinal follow-up.
- PARTICIPATION OF CHILDREN:
- Children under the age of 5 will be excluded from this protocol due to the difficulty of performing pertinent assays in infants and younger children, difficulty distinguishing significance of respiratory infections which occur commonly in younger children, and the lack of adequate facilities and equipment for management of children younger than 2 years.
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR), 800-411-1222 ext. TTY dial 711, ccopr@nih.gov
About this study
Bronchiectasis is a chronic inflammatory and muco-obstructive condition that results in abnormal dilation of the airways, decline in lung function, frequent exacerbations, and chronic respiratory tract infections. Cystic fibrosis (CF) is the most well studied genetic condition that causes bronchiectasis. Tremendous strides made in understanding the mechanisms of CF have led to a multitude of novel treatment strategies that have revolutionized the field. In contrast, the mechanisms of other causes for bronchiectasis remain undetermined and the disease-specific treatments for cystic fibrosis are not effective for non-CF bronchiectasis. Bronchiectasis not caused by CF carries a substantial socioeconomic burden, is a lifelong condition with no cure and, in most cases, has no identifiable cause. Prioritizing research in this important respiratory disease is essential because it affects individuals from children through older adulthood.
Background:
Read more
This protocol is aimed at exploring underlying causes and mechanisms for the development of bronchiectasis. Participants will undergo testing based on their individual clinical presentations to identify underlying causative factors. Additionally, some patients will be followed over time to better understand the natural history of evolving bronchiectasis and to assess the effect of management strategies on the course of the disease and on their chronic infections like the nontuberculous mycobacteria. As potential novel therapies develop, participants may be offered the opportunity to participate in clinical trials.
Objectives:
The primary objective of this study is to identify causes and/or underlying conditions associated with bronchiectasis.
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....
Read the detailed description
Bronchiectasis is a chronic inflammatory and muco-obstructive condition that results in abnormal dilation of the airways, decline in lung function, frequent exacerbations, and chronic respiratory tract infections. Cystic fibrosis (CF) is the most well studied genetic condition that causes bronchiectasis. Tremendous strides made in understanding the mechanisms of CF have led to a multitude of novel treatment strategies that have revolutionized the field. In contrast, the mechanisms of other causes for bronchiectasis remain undetermined and the disease-specific treatments for cystic fibrosis are not effective for non-CF bronchiectasis. Bronchiectasis not caused by CF carries a substantial socioeconomic burden, is a lifelong condition with no cure and, in most cases, has no identifiable cause. Prioritizing research in this important respiratory disease is essential because it affects individuals from children through older adulthood, including those covered by Medicare.
- Keywords
- Variant Cystic Fibrosis, Bronchopulmonary Mycosis, Airway Infection Susceptibility, Chronic Respiratory Infection, Primary Ciliary Dyskinesia, Natural History
- Medical subject headings
- Bronchiectasis, Cystic Fibrosis, Autoimmune Diseases, Common Variable Immunodeficiency, Ciliary Motility Disorders
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| 1 | Children age 5 years and above and adults with chronic or recurring respiratory infections and/or bronchiectasis |
What is being measured
The primary outcome is the main question the study is designed to answer.
The primary objective of this study is to identify causes and/or underlying conditions associated with bronchiectasis.
We will document the presence of underlying associated conditions such as; a. Variant cystic fibrosis through assessment of sweat chloride.b. Primary ciliary dyskinesia through assessment of nasal nitric oxide production, mucosal scrapes for transmission electron microscopy of ciliary ultrastructure.c. Primary immunodeficiency through measurement of quantitative immunoglobulins, specific antigen responses , and lymphocyte phenotyping. d. Allergic bronchopulmonary mycosis through measurement of total immunoglobulin E (IgE), fungal serologies, and skin test responses.e. Autoimmune disease by screening for associated conditions such as inflammatory bowel disease or rheumatoid arthritis.f. Alpha-1 antitrypsin deficiency by assessment of protein levels, phenotype, and gene type.g. Other diagnoses
Measured: Baseline, annually, and optional exacerbation visits, or until they no longer wish to return.
Secondary outcomes (2)
2) Monitor a cohort of bronchiectasis patients to better understand factors associated with progression of disease and to assess outcomes of management and therapeutic strategies
a.<TAB>Patient Global Impression of Severity (PGI-S) b.<TAB>Quality of Life Questionnaire-Bronchiectasis (QOL-B)c.<TAB>Chronic Airways Assessment Test (CAAT) d.<TAB>Bronchiectasis Health Questionnaire (BQH)e.<TAB>Child Chronic Cough-Specific Quality of Life (CC-QOL (7-17 years))f.<TAB>Parent Cough-Specific Quality of Life (PC-QOL Parent completed (2-18 years))g.<TAB>Six-minute walk h.<TAB>Chest CT i.<TAB>Lung function: FEV1%predicted, FVC% predicted, response to bronchodilator, lung volumes, LCI, and DLCOj.<TAB>Lower airway microbiology, as well as novel biomarkers of mycobacteria, antimicrobial resistance and microbial changes on the progression of bronchiectasis. k.<TAB>Inflammatory markers
Measured: Baseline, annually, and optional exacerbation visits. or until they no longer wish to return.
1) Define mechanisms and pathophysiology for the development and progression of bronchiectasis
We will also assess the following mechanisms and clinical outcomes:1) Systemic immune, and/or epithelial surface defense mechanisms involved in airway infection susceptibility and/or development of bronchiectasis through the:a.<TAB>Collection and storage of blood, sputum, and stoolb.<TAB>Use and storage of other relevant biologic specimens, such as bronchoalveolar lavage fluid or *endobronchial biopsy specimens of respiratory epithelium
Measured: Baseline, annually, and optional exacerbation visits. or until they no longer wish to return.
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted22 Jul 2009 (estimated)
- Study start1 Aug 2009
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for BronchiectasisStudies of the same condition, open and under way.
- More trials run by National Heart, Lung, and Blood Institute (NHLBI)The sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Prospective
- Enrolment
- 900 participants (estimated)
Sponsor and investigators
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Pamela J McShane, M.D., Principal investigator, National Heart, Lung, and Blood Institute (NHLBI)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00943514
- Sponsor's protocol ID
- 090172
- Other IDs
- 09-H-0172
- Status last verified
- 28 Oct 2026
- First submitted
- 21 Jul 2009
- Last updated
- 5 Nov 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Yes: Refer to the DMSP
Publications
Lunich A, Radtke AJ, Williams M, Stern JM, Barber DL, Germain RN, Anidi IU. Optimized Workflow for Iterative Bleaching Extends Multiplexity Imaging of Highly Autofluorescent Clinical Samples. J Vis Exp. 2025 Jul 11;(221). doi: 10.3791/67980.
PubMed 40720370
Study at a glance
From the registry record, last updated by the sponsor on 5 Nov 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Natural History of Bronchiectasis
- ClinicalTrials.gov ID
- NCT00943514
- Acronym / sponsor ID
- 090172
- Keywords
- Variant Cystic Fibrosis, Bronchopulmonary Mycosis, Airway Infection Susceptibility, Chronic Respiratory Infection, Primary Ciliary Dyskinesia, Natural History
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 900 participants (estimated)
- Lead sponsor
- National Heart, Lung, and Blood Institute (NHLBI)US National Institutes of Health
- Study start
- 1 Aug 2009 (actual)
- First posted
- 22 Jul 2009 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Bronchiectasis is a chronic inflammatory and muco-obstructive condition that results in abnormal dilation of the airways, decline in lung function, frequent exacerbations, and chronic respiratory tract infections. Cystic fibrosis (CF) is the most well studied genetic condition that causes bronchiectasis. Tremendous strides made in understanding the mechanisms of CF have led to a multitude of novel treatment strategies that have revolutionized the field. In contrast, the mechanisms of other causes for bronchiectasis remain undetermined and the disease-specific treatments for cystic fibrosis are not effective for non-CF bronchiectasis. Bronchiectasis not caused by CF carries a substantial socioeconomic burden, is a lifelong condition with no cure and, in most cases, has no identifiable cause. Prioritizing research in this important respiratory disease is essential because it affects individuals from children through older adulthood.
Background:
This protocol is aimed at exploring underlying causes and mechanisms for the development of bronchiectasis. Participants will undergo testing based on their individual clinical presentations to identify underlying causative factors. Additionally, some patients will be followed over time to better understand the natural history of evolving bronchiectasis and to assess the effect of management strategies on the course of the disease and on their chronic infections like the nontuberculous mycobacteria. As potential novel therapies develop, participants may be offered the opportunity to participate in clinical trials.
Objectives:
The primary objective of this study is to identify causes and/or underlying conditions associated with bronchiectasis.
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 is a chronic inflammatory and muco-obstructive condition that results in abnormal dilation of the airways, decline in lung function, frequent exacerbations, and chronic respiratory tract infections. Cystic fibrosis (CF) is the most well studied genetic condition that causes bronchiectasis. Tremendous strides made in understanding the mechanisms of CF have led to a multitude of novel treatment strategies that have revolutionized the field. In contrast, the mechanisms of other causes for bronchiectasis remain undetermined and the disease-specific treatments for cystic fibrosis are not effective for non-CF bronchiectasis. Bronchiectasis not caused by CF carries a substantial socioeconomic burden, is a lifelong condition with no cure and, in most cases, has no identifiable cause. Prioritizing research in this important respiratory disease is essential because it affects individuals from children through older adulthood, including those covered by Medicare.
Study design
- Observational model
- Other
- Time perspective
- Prospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| 1 | – | –Children age 5 years and above and adults with chronic or recurring respiratory infections and/or bronchiectasis |
Who may qualify
- Ages
- 5 to 100 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
- Study population
- Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections and/or bronchiectasis will be eligible for participation. Relatives including 5 years and above.
Key inclusion criteria (12 listed)
- The research that is conducted on this protocol is regulated by Health and Human Services, and in compliance with federal guidelines Title 45 Code of Federal Regulations, Part 46, Subpart D. The groups of children that may be included are:
- Affected minors: Bronchiectasis may have early onset and a significant impact on the lives of these patients. It is important to include this population in our study in order to understand the natural history and progression of this condition in children as they often have physiological differences as compared to adult patients. Affected minors may undergo testing that will be tailored to their specific clinical presentation.
- Minors that are related to affected individuals: Children that are relatives of index patients may not be affected or show symptoms. It is important for relatives including minor relatives to be enrolled in our study, in order to help us identify the possible conditions like pre-bronchiectasis to better understand the mechanism of the disease progression. Unaffected child relatives may only undergo tests that are no greater than minimal risk.
- Rational for inclusion of affected pregnant and or nursing women: Pregnant and or nursing women including pregnant minors may be included in accordance with Federal Regulations at Subpart B of 45 CFR 46. Bronchiectasis-related symptoms and respiratory status may change during pregnancy. Including pregnant participants in this observational natural history protocol will allow the study to capture longitudinal changes in clinical status and other relevant data during pregnancy and the postpartum period.
- Study procedures conducted during pregnancy and breastfeeding will be limited to those determined to pose no more than minimal risk to the participant and fetus or breastfeeding infant. Procedures considered greater than minimal risk -including computed tomography scans, bronchoscopy with bronchoalveolar lavage and endobronchial biopsy, and research blood collection- will not be performed during pregnancy or breastfeeding. These procedures may be completed after the participant is no longer pregnant and, when applicable, has discontinued breastfeeding, in accordance with the protocol and investigator judgment.
Key exclusion criteria (5 listed)
- <!-- -->
- Children under the age of 5 will be excluded from this protocol due to the difficulty of performing pertinent assays in infants and younger children, difficulty distinguishing significance of respiratory infections which occur commonly in younger children, and the lack of adequate facilities and equipment for management of children younger than 2 years.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
The primary objective of this study is to identify causes and/or underlying conditions associated with bronchiectasis.
Time frame: Baseline, annually, and optional exacerbation visits, or until they no longer wish to return.
We will document the presence of underlying associated conditions such as; a. Variant cystic fibrosis through assessment of sweat chloride.b. Primary ciliary dyskinesia through assessment of nasal nitric oxide production, mucosal scrapes for transmission electron microscopy of ciliary ultrastructure.c. Primary immunodeficiency through measurement of quantitative immunoglobulins, specific antigen responses , and lymphocyte phenotyping. d. Allergic bronchopulmonary mycosis through measurement of total immunoglobulin E (IgE), fungal serologies, and skin test responses.e. Autoimmune disease by screening for associated conditions such as inflammatory bowel disease or rheumatoid arthritis.f. Alpha-1 antitrypsin deficiency by assessment of protein levels, phenotype, and gene type.g. Other diagnoses
2 secondary outcomes
2) Monitor a cohort of bronchiectasis patients to better understand factors associated with progression of disease and to assess outcomes of management and therapeutic strategies
Time frame: Baseline, annually, and optional exacerbation visits. or until they no longer wish to return.
1) Define mechanisms and pathophysiology for the development and progression of bronchiectasis
Time frame: Baseline, annually, and optional exacerbation visits. or until they no longer wish to return.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 5 Nov 2026.
- Derived from this study: PubMed 40720370Lunich A, Radtke AJ, Williams M, Stern JM, Barber DL, Germain RN, Anidi IU. Optimized Workflow for Iterative Bleaching Extends Multiplexity Imaging of Highly Autofluorescent Clinical Samples. J Vis Exp. 2025 Jul 11;(221). doi: 10.3791/67980.
- NIH Clinical Center Detailed Web PageLink provided by the sponsor.
- Individual participant dataSharing planned: Yes. Refer to the DMSP
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Pamela J McShane, M.D. principal investigator
National Heart, Lung, and Blood Institute (NHLBI)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Bronchiectasis and Cystic Fibrosis, run by National Heart, Lung, and Blood Institute (NHLBI) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 12 inclusion and 5 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 5 Nov 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Chevalia J Robinson, R.N. ((301) 496-3973, robinsoc1@nih.gov), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
The primary objective of this study is to identify causes and/or underlying conditions associated with bronchiectasis. (time frame: Baseline, annually, and optional exacerbation visits, or until they no longer wish to return.). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00943514, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 5 Nov 2026.