Understanding the natural history of bronchiectasis

Natural History of Bronchiectasis

This study looks at how bronchiectasis develops over time in people with chronic lung infections, especially focusing on genetic and immune factors that might make them more prone to these infections.

Quick facts

• Study type: Observational
• Enrollment: 900 (estimated)
• Ages: 5 Years to 100 Years
• Sex: All
• Sponsor: National Institutes of Health Clinical Center (CC) (NIH)
• Locations: 1 site (Bethesda, Maryland)
• Trial ID: NCT00943514 on ClinicalTrials.gov

What this trial studies

This observational study aims to investigate the natural history of bronchiectasis, focusing on identifying inherited and immune factors that contribute to chronic respiratory infections. Participants aged 5 years and older with a history of chronic or recurring respiratory infections will be enrolled, including those with cystic fibrosis and other immune defects for comparative purposes. The study will collect various biological samples to elucidate mechanisms of infection susceptibility and improve understanding of the disease's progression.

Who should consider this trial

Good fit: Ideal candidates include individuals aged 5 and older with chronic or recurring respiratory infections, particularly those with non-cystic fibrosis bronchiectasis.

Not a fit: Patients without chronic respiratory infections or those who do not meet the age criteria may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to better-targeted treatments for bronchiectasis and improve patient outcomes.

How similar studies have performed: While there have been studies on cystic fibrosis, this approach to understanding non-CF bronchiectasis is relatively novel and less explored.

Eligibility criteria

* PARTICIPANT INCLUSION CRITERIA:

  1. Children age 5 years and above and adults referred to the NIH with chronic or recurring respiratory infections will be eligible for participation.
  2. Males and females will be considered without regard to race or ethnicity or upper age limit.
  3. Emphasis will be primarily on non CF bronchiectasis for elucidation of mechanisms of 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.
  4. Subjects must have a primary or referring physician
  5. Subjects must be willing to have samples stored

INCLUSION CRITERIA FOR RELATIVES:

As a part of this protocol we may obtain blood, sputum, urine, or buccal swabs from some blood relatives of patients on the study, with the hope of isolating and characterizing the primary host defense defect(s) or genetic links responsible for airway infection susceptibility and/or bronchiectasis seen within families. Male and female relatives will be accepted without limitation due to age. These relatives may have pertinent disease-related history obtained, but will neither receive treatment nor have any other protocol procedures done unless they are enrolled on the study.

PARTICIPANT EXCLUSION CRITERIA:

1. We wish to avoid enrolling subjects, especially children, who have common respiratory problems (aeroallergen sensitivity, asthma, gastric esophageal reflux) that are not associated with an underlying abnormality in host defenses. Evidence of significance of chronic or recurring infections suggestive of an underlying airway surface or systemic host defense defect should be documented by one or more of the following: a) sinus or lower airway cultures positive for bacterial, fungal, or mycobacterial pathogens characteristic of these defects; or b) radiographic evidence of sinusitis with mucosal thickening and/or air-fluid levels; or c) radiographic evidence of bronchiectasis; d) severity of otitis media requiring placement of tympanic membrane pressure equalization tubes; e) severity of sinusitis requiring surgical intervention.
2. Patients who are unable or unwilling to provide informed consent either directly or via appropriately designated surrogate.
3. 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.

Where this trial is running

Bethesda, Maryland

• National Institutes of Health Clinical Center — Bethesda, Maryland, United States (Recruiting)

Study contacts

• Principal investigator: Kevin P Fennelly, M.D. — National Heart, Lung, and Blood Institute (NHLBI)
• Study coordinator: Chevalia J Robinson, R.N.

Conditions Bronchiectasis; Cystic Fibrosis; Autoimmune Disease; Common Variable Immunodeficiency; Variant Cystic Fibrosis; Bronchopulmonary Mycosis; Airway Infection Susceptibility; Chronic Respiratory Infection