Bronchiectasis
Pulmonary and Critical Care Medicine · Airways Disease 🎯 Practice these cards
Bronchiectasis is defined by ____ airway dilation due to destruction of airway wall architecture; the most common morphologic form is the ____ (tubular) type.
Bronchiectasis is a chronic suppurative lung disease with IRREVERSIBLE airway dilation from destruction of airway wall architecture (elastin, smooth muscle, cartilage). Morphologic forms: cylindrical/tubular (MOST COMMON), varicose, and cystic. Disease may be focal (obstruction) or diffuse (systemic/infectious process).
In the vicious cycle hypothesis, impaired mucociliary clearance permits bacterial ____ (the signature organism is ____), which drives chronic neutrophilic inflammation and progressive airway-wall destruction.
Pathogenesis follows a "vicious cycle": impaired mucociliary clearance + susceptibility to infection → bacterial COLONIZATION (Pseudomonas aeruginosa is especially adept at adhering to damaged airways and evading defenses) → chronic NEUTROPHILIC inflammation → protease/ROS-mediated airway-wall destruction → mucus stasis and further obstruction → more infection. Bacterial products themselves impair ciliary clearance. Molecular endotypes with distinct microbiome signatures correlate with exacerbation risk.
The most common identifiable causes of bronchiectasis are cystic fibrosis, aspiration, ____, and connective tissue diseases; even after full evaluation, more than ____ of cases are idiopathic.
Most common identifiable causes: cystic fibrosis, chronic ASPIRATION, IMMUNODEFICIENCY (CVID, HIV), and CONNECTIVE TISSUE DISEASES (RA, Sjögren's, IBD). Others: airway obstruction (tumor, foreign body, COPD), ABPA, primary ciliary dyskinesia/Kartagener, Mounier-Kuhn and Williams-Campbell syndromes, Young syndrome, TB/NTM/recurrent pneumonia. Even after rigorous evaluation, MORE THAN HALF of cases are labeled idiopathic (25–50% in referral series).
Upper-lobe bronchiectasis suggests ____ or postradiation fibrosis; mid-lung predominance suggests ____ or ciliary dyskinesia; central-airway disease suggests ____.
UPPER-lobe predominance: cystic fibrosis, postradiation fibrosis. LOWER-lobe predominance: chronic aspiration (e.g., scleroderma), traction bronchiectasis (IPF), immunodeficiency (hypogammaglobulinemia). MID-lung predominance: NTM (MAC classically in nonsmoking women >50), ciliary dyskinesia. CENTRAL-airway predominance: ABPA, Mounier-Kuhn (tracheobronchomegaly), Williams-Campbell (cartilage deficiency).
The two cardinal HRCT criteria for bronchiectasis are an airway diameter ____ than its accompanying vessel (the "____ sign") and lack of distal airway ____.
High-resolution chest CT is the test of choice (plain films are insensitive — "tram tracks" may be seen). Diagnostic findings: airway diameter GREATER than the accompanying vessel ("signet-ring sign"; consensus airway-to-artery ratio ≥1.5) + LACK OF DISTAL TAPERING (airways visible within 1 cm of the pleura). Supporting: bronchial wall thickening, tree-in-bud inspissated secretions, bronchial-wall cysts.
The 2025 EMBRACE consensus defines bronchiectasis by chronic cough/sputum plus HRCT changes regardless of ____, repositioning CF bronchiectasis as a ____ of bronchiectasis.
The 2025 EMBRACE Delphi consensus (Lancet Respir Med 2025;13:951-964) defines bronchiectasis by chronic COUGH and SPUTUM production plus characteristic HRCT changes, REGARDLESS OF ETIOLOGY — CF bronchiectasis is repositioned as a subtype of bronchiectasis, not a separate entity. The earlier Aliberti 2022 consensus required ≥1 radiologic criterion (airway-artery ratio ≥1.5, lack of tapering, peripheral airway visibility) plus ≥2 clinical features (daily cough, daily sputum, exacerbation history).
The core etiologic workup includes sputum cultures (bacterial, AFB, fungal), ____ levels and HIV testing, and autoimmune serologies; FOCAL bronchiectasis requires ____ to exclude an obstructing lesion.
Every newly diagnosed patient needs a search for a treatable cause: comprehensive history (childhood infections e.g. pertussis, family history), sputum Gram stain/culture + AFB and fungal studies, immunoglobulin levels + HIV testing, connective-tissue-disease serologies (e.g., rheumatoid factor). SELECTED patients: sweat chloride/CFTR genetics (CF), nasal/respiratory brush or biopsy (ciliary dysfunction), α1-antitrypsin level. No pathogen found → consider bronchoscopy with BAL. FOCAL disease → bronchoscopy to exclude obstruction.
Bronchiectasis exacerbations may lack fever and new infiltrates; when no prior culture data exist, empiric therapy with a ____ is recommended to ensure ____ coverage.
Exacerbation evidence: change in sputum VOLUME, VISCOSITY, or PURULENCE; increased cough; wheezing; dyspnea; hemoptysis; or decline in lung function. May be hard to separate from baseline — and FEVER and NEW INFILTRATES may be ABSENT. Therapy is guided by routine sputum + AFB cultures; empiric therapy from prior culture data; no prior data → FLUOROQUINOLONE for Pseudomonas coverage. Duration typically 7–10 days, up to 14. Most common isolates: Haemophilus influenzae and P. aeruginosa.
Before chronic macrolide prophylaxis you must exclude ____ infection and check the ____ interval on ECG; the benefit is attributed to non-antimicrobial ____ effects.
Randomized trials (BAT, BLESS, EMBRACE) show 6–12 months of azithromycin or erythromycin reduces exacerbation rates, mucus production, and lung-function decline — via NON-antimicrobial anti-inflammatory and anti-biofilm effects. BEFORE starting: (1) EXCLUDE NTM infection (risk of macrolide-resistant NTM), (2) ECG for QT prolongation. Caveat: increases macrolide resistance in commensals → reserve for high-morbidity patients (e.g., ≥3 exacerbations/year).
Dornase alfa is a routine mucolytic in ____ bronchiectasis but should NOT be used in ____ bronchiectasis (lack of efficacy + potential harm).
Dornase alfa (recombinant DNase) is routinely recommended in CF-related bronchiectasis but is NOT recommended in NON-CF bronchiectasis: trials showed lack of efficacy and signals of HARM (more exacerbations, worse FEV1). The 2023 ERS airway clearance statement reinforces this. Non-CF airway clearance = hydration, mucolytics, hypertonic saline, bronchodilators, chest physiotherapy (postural drainage, oscillatory PEP, high-frequency chest-wall oscillation vest), exercise/pulmonary rehab; ERS 2023 recommends teaching an active cycle of breathing technique for ≥3 exacerbations/year or ≥1 hospitalization.
ERS 2025 conditionally recommends inhaled antibiotics (preferably ____ or colistimethate) for ≥3 exacerbations/year; the phase 3 trials of inhaled ____ were negative.
The 2025 ERS statement CONDITIONALLY recommends inhaled antibiotics for adults with non-CF bronchiectasis with ≥3 exacerbations/year or ≥1 hospitalization despite optimized care — preferably inhaled TOBRAMYCIN or COLISTIMETHATE for Pseudomonas. The phase 3 EMBRACE-1/2 trials of inhaled AZTREONAM (NEJM Evid 2026;5(3)) were NEGATIVE (no reduction in annualized exacerbation rate), supporting the tobramycin/colistin preference. Inhaled agents aim to lower airway microbial load while limiting systemic toxicity.
Brensocatib, a first-in-class oral ____ (cathepsin C) inhibitor, reduced the annualized exacerbation rate by ____ in the ASPEN phase 3 trial.
Brensocatib is a first-in-class oral DIPEPTIDYL PEPTIDASE-1 (cathepsin C) inhibitor that reduces neutrophil serine protease activity. ASPEN phase 3 (NEJM 2025;393:1961-1972; n=1689): the 25-mg dose reduced the annualized exacerbation rate by 21% and prolonged time to first exacerbation (HR 0.76) over 52 weeks; well tolerated (hyperkeratosis/periodontitis numerically higher). FDA decision expected by MARCH 2026 — would be the FIRST approved therapy for non-CF bronchiectasis.
In massive hemoptysis, position the patient ____-side down to protect the nonbleeding lung; the usual definitive intervention is ____.
Recurrent infection erodes superficial mucosal vessels → hemoptysis that can be life-threatening. Management: stabilize the airway (intubation if needed), PROTECT THE NONBLEEDING LUNG (position bleeding-side down), identify the source, and perform BRONCHIAL ARTERY EMBOLIZATION; surgery if bleeding is uncontrolled.
Chronic ____ colonization predicts a worse prognosis; FEV1 in non-CF bronchiectasis declines by about ____ mL per year, similar to COPD.
Outcomes vary with etiology, comorbidity, exacerbation frequency, and colonizing pathogen — chronic PSEUDOMONAS colonization predicts a WORSE course. FEV1 declines ~50–55 mL/YEAR in non-CF bronchiectasis (similar to COPD) versus 20–30 mL/year in healthy adults. Prevention: vaccination (influenza, pneumococcal, COVID-19, RSV), smoking cessation, immunoglobulin replacement for the immunodeficient. Localized refractory disease → surgical resection; advanced disease → transplant.
True NTM infection is supported by ≥2 positive ____ cultures or ≥1 positive ____ culture; macrolide-susceptible MAC is treated with a macrolide plus rifampin and ____.
NTM (most commonly MAC) can colonize OR infect. Diagnostic support: ≥2 positive sputum cultures, OR ≥1 positive BAL culture, OR biopsy with compatible histopathology + one positive culture. Macrolide-susceptible MAC in HIV-negative patients: MACROLIDE + RIFAMPIN + ETHAMBUTOL, with macrolide susceptibility testing. NTM classically affects mid-lung fields in nonsmoking women >50. This is why NTM must be excluded before chronic macrolide monotherapy.
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