What is the primary definition of bronchiectasis?
The permanent and irreversible dilation, or ectasia, of the large airways or bronchi.
What diagnostic imaging modality is considered the most accurate for diagnosing bronchiectasis?
High-resolution computed tomography (HRCT).
What are the three morphological classifications of bronchiectasis based on radiographic patterns?
Cylindrical, varicoid (or varicose), and cystic.
The proposed pathogenic model in bronchiectasis involving airway epithelial dysfunction, chronic infections, inflammation, and airway injury is known as the _____.
vicious vortex (or vicious cycle)
What is the main inflammatory cell type that infiltrates the airways in bronchiectasis, releasing proteolytic enzymes like neutrophil elastase?
Neutrophils.
According to the ERS guidelines, what is the minimum bundle of etiological tests suggested for all adults with a new diagnosis of bronchiectasis?
Differential blood count, serum immunoglobulins (IgG, IgA, IgM), and testing for allergic bronchopulmonary aspergillosis (ABPA).
What is the primary clinical manifestation that drives progression, healthcare costs, and mortality in bronchiectasis?
Exacerbations.
According to ERS guidelines, long-term antibiotic treatment should be considered for adults with bronchiectasis who have how many exacerbations per year?
Three or more exacerbations per year.
For a bronchiectasis patient with ≥3 exacerbations/year and chronic P. aeruginosa infection, what is the suggested first-line long-term antibiotic treatment according to ERS guidelines?
An inhaled antibiotic.
INDICATIONS to long term ABO (provided with >3 exacerbations/year):
exacerbation despite inhaled ABO: MACROLIDES (AZ, Erythromycin)
For a bronchiectasis patient with ≥3 exacerbations/year not infected with P. aeruginosa, what class of oral antibiotics is suggested as the first-line long-term treatment by ERS guidelines?
Macrolides (e.g., azithromycin, erythromycin).
What is the term for bronchiectasis for which no known predisposing cause is identified after a thorough evaluation?
Idiopathic bronchiectasis.
Which mucoactive agent, an inhaled dry powder, has shown promise in airway clearance for bronchiectasis patients but may be difficult to tolerate?
Mannitol.
What was the outcome of trials using recombinant human deoxyribonuclease (rhDNase) in patients with idiopathic bronchiectasis?
It failed to reduce exacerbations and showed a trend toward more frequent and severe exacerbations.
In bronchiectasis, transmural inflammation causes damage, making airways susceptible to chronic colonization by microorganisms such as Aspergillus, NTM, and particularly _____.
Pseudomonas aeruginosa
The Bronchiectasis Severity Index (BSI) and the FACED score are tools used for what purpose?
To assess long-term prognosis and predict mortality and hospitalization risk in non-CF bronchiectasis.
What does the ‘F’ in the FACED prognostic score for bronchiectasis stand for?
FEV1 (Forced Expiratory Volume in 1 second).
What is the most common immunodeficiency associated with bronchiectasis?
Common Variable Immunodeficiency (CVID).
The diagnosis of Common Variable Immunodeficiency (CVID) is characterized by reduced serum levels of IgG, IgA, and/or IgM, along with what other key finding?
Reduced or absent antibody production in response to a specific antigen challenge (vaccination).
What is the classic triad of symptoms associated with Kartagener syndrome, a subtype of Primary Ciliary Dyskinesia (PCD)?
Situs inversus, chronic sinusitis, and bronchiectasis.
What is the diagnostic criterion for Allergic Bronchopulmonary Aspergillosis (ABPA) in a patient with asthma or CF, regarding total serum IgE levels?
A total serum IgE level greater than 1000 IU/mL.
When is surgical resection considered a management option for bronchiectasis?
In carefully selected patients with severe, localized disease refractory to medical therapy or for complications like life-threatening hemoptysis.
What is the general recommendation from the ERS guidelines regarding the use of inhaled corticosteroids (ICS) in bronchiectasis patients who do not have a clear history of asthma or COPD?
There is insufficient evidence to recommend for or against their use, and further trials are needed.
According to epidemiological data, the prevalence of non-CF bronchiectasis increases substantially with _____, reaching over 800 cases per 100,000 for ages ≥75 years.
age
The ERS guidelines suggest that in bronchiectasis patients with chronic P. aeruginosa infection who cannot tolerate inhaled antibiotics, what is the next therapeutic option?
Long-term treatment with macrolides.