An observational study in Bronchiectasis, Cystic Fibrosis and Autoimmune Disease, sponsored by National Heart, Lung, and Blood Institute (NHLBI). Recruiting at 1 site in United States. Open to participants aged 5 Years to 100 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2026-11-09.
Sponsored by National Heart, Lung, and Blood Institute (NHLBI) · Observational
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:
Design:
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.
404 studies on the registry are indexed under Bronchiectasis; 125 are open to participants now.
This study's planned enrollment of 900 is above the median of 160 across 137 observational studies indexed under Bronchiectasis.
Browse Bronchiectasis studies →National Heart, Lung, and Blood Institute (NHLBI) is the lead sponsor of 1,143 studies on the registry; 71 are open to participants now.
Of its 58 completed or terminated interventional studies of FDA-regulated products, 51 (88%) have results posted.
Counted across the registry records on this site, refreshed daily.
The criteria were revised on the registry on Oct 30, 2026. See what changed
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.
INCLUSION CRITERIA:
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.
INCLUSION OF VULNERABLE PARTICIPANTS
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 , 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.
EXCLUSION CRITERIA:
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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.
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
The human immunodeficiency virus, which weakens the immune system.
Many studies now accept people whose HIV is controlled on treatment, judged by the viral load and the CD4 count. Check the limits this study sets.
A biopsy takes a small piece of tissue to examine. “Archival tissue” is a sample already taken, for example at diagnosis, and kept by the hospital.
Whether a stored sample will do depends on when it was taken, how much there is and what the study needs. Ask whether the study accepts your stored sample or needs a new biopsy.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
HIV
The human immunodeficiency virus, which weakens the immune system.
Many studies now accept people whose HIV is controlled on treatment, judged by the viral load and the CD4 count. Check the limits this study sets.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Biopsy and archival tissue
A biopsy takes a small piece of tissue to examine. “Archival tissue” is a sample already taken, for example at diagnosis, and kept by the hospital.
Whether a stored sample will do depends on when it was taken, how much there is and what the study needs. Ask whether the study accepts your stored sample or needs a new biopsy.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Children age 5 years and above and adults with chronic or recurring respiratory infections and/or bronchiectasis
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
Time frame: Baseline, annually, and optional exacerbation visits, or until they no longer wish to return.
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
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
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
Time frame: Baseline, annually, and optional exacerbation visits. or until they no longer wish to return.
Plan to share: Yes — Refer to the DMSP
Supporting information: Study protocol, Statistical analysis plan, Informed consent form
Time frame: Refer to the DMSP
Access criteria: Refer to the DMSP
Started Aug 2009 (actual).
How these figures are worked out, and which studies are left out of them.
From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗
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