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Bronchiectasis

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Overview

Bronchiectasis

Quick Reference

Critical Alerts

  • Massive hemoptysis can occur and requires emergent management
  • Pseudomonas aeruginosa is common - consider antipseudomonal coverage
  • Respiratory failure may develop in severe exacerbations
  • Chronic colonization differs from acute infection - know patient's baseline
  • Non-tuberculous mycobacteria (NTM) may be present - consider if not responding

Key Diagnostics

  • Chest X-ray (may show bronchial wall thickening, "tram tracks")
  • CT chest (gold standard for diagnosis - bronchial dilation)
  • Sputum culture including AFB (guide antibiotic therapy)
  • CBC, CRP (inflammatory markers)
  • ABG/VBG if respiratory distress

Emergency Treatments

  • Oxygen therapy: Target SpO2 88-92% (may have chronic hypercapnia)
  • Antibiotics: Empiric based on prior cultures; cover Pseudomonas if colonized
  • Bronchodilators: Nebulized salbutamol and ipratropium
  • Airway clearance: Physiotherapy, nebulized hypertonic saline
  • Hemoptysis management: Tranexamic acid, bronchial artery embolization if massive

Definition

Bronchiectasis is a chronic respiratory disease characterized by irreversible, abnormal dilation of the bronchi with impaired mucociliary clearance, chronic infection, and inflammation. Patients experience recurrent respiratory infections and progressive lung damage.

Pathological Classification

TypeDescription
CylindricalUniform dilation of bronchi (most common)
VaricoseIrregular dilation with beaded appearance
Cystic/SaccularSevere dilation with sac-like structures (most severe)

Epidemiology

  • Prevalence: 50-500 per 100,000 (increasing with CT availability)
  • Age: Can occur at any age; prevalence increases with age
  • Sex: Female predominance in non-CF bronchiectasis
  • Mortality: Increased compared to general population

Etiology

CategoryCauses
Post-infectiousChildhood pneumonia, TB, pertussis, measles
ImmunodeficiencyCommon variable immunodeficiency, HIV, IgG subclass deficiency
GeneticCystic fibrosis, primary ciliary dyskinesia, alpha-1 antitrypsin
AspirationGERD, neurological conditions
AutoimmuneRheumatoid arthritis, Sjögren's syndrome
AllergicABPA (allergic bronchopulmonary aspergillosis)
ObstructionForeign body, tumor
IdiopathicUp to 50% of cases

Pathophysiology

Vicious Cycle Hypothesis

Cole's Vicious Cycle

Initial insult (infection, obstruction, immune defect)
                    ↓
Impaired mucociliary clearance
                    ↓
Bacterial colonization
                    ↓
Chronic inflammation (neutrophils, cytokines)
                    ↓
Airway damage and dilation
                    ↓
Further impairment of clearance
                    ↓
More infection and inflammation (cycle repeats)

Microbiology

Common Colonizing Organisms

OrganismFeatures
Haemophilus influenzaeMost common; non-typeable strains
Pseudomonas aeruginosaAssociated with worse outcomes; difficult to eradicate
Streptococcus pneumoniaeCommon in exacerbations
Moraxella catarrhalisCommon, usually less virulent
Staphylococcus aureusConsider in CF or post-viral
Non-tuberculous mycobacteriaMAC, M. abscessus - difficult to treat
AspergillusABPA, colonization

Complications

  • Recurrent exacerbations
  • Progressive lung function decline
  • Respiratory failure
  • Massive hemoptysis
  • Cor pulmonale
  • Amyloidosis (rare)

Clinical Presentation

Chronic Symptoms

SymptomFrequencyDescription
Chronic productive cough90%+Daily sputum production, often purulent
Dyspnea70-80%Progressive, worse with exacerbations
FatigueCommonRelated to chronic infection
Hemoptysis30-50%Usually mild; can be massive
Chest pain20-30%Pleuritic or related to cough
Recurrent infectionsHallmarkMultiple courses of antibiotics/year

Exacerbation Definition

Acute Exacerbation of Bronchiectasis Deterioration in ≥3 of the following for ≥48 hours:

Physical Examination

General

Respiratory

FindingSignificance
Coarse cracklesSecretions in dilated airways
WheezeAirflow obstruction
Reduced breath soundsSevere disease, consolidation
Increased AP diameterAir trapping

Severity Assessment

BSI (Bronchiectasis Severity Index)


Increased cough
Common presentation.
Increased sputum volume or change in consistency
Common presentation.
Increased sputum purulence
Common presentation.
Increased dyspnea
Common presentation.
Increased fatigue
Common presentation.
Hemoptysis (new or increased)
Common presentation.
Fever
Common presentation.
Red Flags (Life-Threatening)

Critical Presentations

Red FlagConcernAction
Massive hemoptysis (>00mL/24h)Bronchial artery ruptureEmergent bronchial artery embolization
Respiratory failure (SpO2 <88%)Severe exacerbationICU, NIV/intubation consideration
SepsisSevere infectionSepsis protocol, broad antibiotics
New oxygen requirementSignificant declineAdmission, workup
Confusion/altered consciousnessHypercapnia, sepsisABG, ICU consideration
Persistent fever despite antibioticsResistant organism, abscessCT, consider NTM/fungi

Massive Hemoptysis Management

Immediate Actions:
1. Position bleeding lung down (if known)
2. Protect airway - suction
3. Large-bore IV access
4. Type and crossmatch
5. Tranexamic acid 1g IV
6. Urgent CXR (lateralize source)
7. Call for help - ICU, interventional radiology, thoracic surgery

Definitive Treatment:
- Bronchial artery embolization (first-line)
- Bronchoscopy for localization
- Surgery if embolization fails

Differential Diagnosis

Conditions with Similar Presentations

ConditionDistinguishing Features
COPDSmoking history, emphysema on CT, no bronchial dilation
AsthmaReversible obstruction, no structural changes
Chronic bronchitisSmoking related, no bronchiectasis on CT
TuberculosisAFB positive, cavitary lesions
Lung cancerMass on imaging, hemoptysis, weight loss
Interstitial lung diseaseRestrictive pattern, fibrosis on CT
Cystic fibrosisYounger onset, pancreatic insufficiency, sweat test

Causes of Chronic Productive Cough

  • Bronchiectasis
  • COPD
  • Chronic sinusitis with post-nasal drip
  • GERD-related cough
  • ACE inhibitor-related cough
  • Lung cancer

Diagnostic Approach

Clinical Assessment

Key History

  • Duration and characteristics of symptoms
  • Sputum volume and color daily vs during exacerbation
  • Prior cultures and antibiotic sensitivities
  • Exacerbation frequency
  • Prior hospitalizations
  • Known colonizing organisms
  • Baseline pulse oximetry and FEV1

Laboratory Studies

TestPurposeFindings
Sputum cultureGuide antibiotic therapyMay grow colonizing organisms
AFB smear/cultureNTM, TBRule out mycobacterial disease
CBCInfection, anemiaLeukocytosis, anemia of chronic disease
CRPInflammatory markerElevated in exacerbation
ABG/VBGRespiratory failureHypoxemia, hypercapnia
ProcalcitoninBacterial infectionMay help guide antibiotics

Imaging

Chest X-ray

  • May be normal in mild disease
  • "Tram track" sign (thickened bronchial walls)
  • Ring shadows (dilated bronchi en face)
  • Air-fluid levels in dilated bronchi
  • Consolidation if infection

High-Resolution CT (HRCT) - Gold Standard

FindingDescription
Bronchial dilationInternal diameter > adjacent artery (signet ring)
Lack of taperingBronchi visible in outer third of lung
Bronchial wall thickening>mm
Tree-in-bud opacitiesSmall airway disease
Mucus pluggingAir-fluid levels in airways

Pulmonary Function Tests

  • Often obstructive pattern
  • FEV1/FVC <0.7
  • Reduced FVC in advanced disease
  • Air trapping (increased RV/TLC)

Treatment

Acute Exacerbation Management

Antibiotic Selection

Colonization StatusEmpiric RegimenDuration
No prior culturesAmoxicillin-clavulanate 875/125mg PO BID14 days
H. influenzaeAmoxicillin-clavulanate or doxycycline14 days
PseudomonasCiprofloxacin 750mg PO BID or IV antipseudomonal14 days
MRSATMP-SMX or doxycycline14 days
Severe/hospitalizedIV piperacillin-tazobactam or ceftazidime + aminoglycoside14 days

Key Principle: Use prior culture data to guide therapy

Bronchodilators

  • Nebulized salbutamol 2.5-5mg q4-6h
  • Nebulized ipratropium 500mcg q6h
  • Consider if wheeze or airflow obstruction

Airway Clearance

  • Chest physiotherapy
  • Active cycle of breathing technique
  • Nebulized hypertonic saline (3-7%) - loosens secretions
  • Mucolytics (dornase alfa only in CF; others unproven)

Oxygen Therapy

  • Target SpO2 88-92% (may have chronic hypercapnia)
  • Avoid high-flow oxygen if CO2 retainer
  • Check ABG if concern for hypercapnia

Hemoptysis Management

Minor Hemoptysis (<100mL/24h)

  • Reassurance
  • Treat underlying infection
  • Consider tranexamic acid 500mg PO TID
  • Avoid chest physiotherapy during active bleeding

Moderate/Massive Hemoptysis

  • As per red flag management above
  • Tranexamic acid 1g IV
  • Bronchial artery embolization
  • Rigid bronchoscopy for airway control if needed

Non-Invasive Ventilation

Indications

  • Hypercapnic respiratory failure
  • pH <7.35 with PaCO2 >45 mmHg
  • Severe respiratory distress

Settings

  • IPAP 12-20 cmH2O
  • EPAP 4-6 cmH2O
  • Titrate to patient comfort and ABG response

Chronic Management (Outpatient)

InterventionPurpose
Airway clearance (daily)Reduce sputum stasis
Inhaled bronchodilatorsImprove airflow, aid clearance
Pulmonary rehabilitationImprove exercise tolerance
Macrolide prophylaxisReduce exacerbations (azithromycin 250mg 3x/week)
VaccinationsInfluenza, pneumococcal, COVID-19
Nebulized tobramycinPseudomonas eradication/suppression

Disposition

Admission Criteria

  • Hypoxia requiring supplemental oxygen
  • Severe exacerbation with respiratory distress
  • Failure of outpatient antibiotics
  • Inability to manage at home
  • Significant hemoptysis
  • Need for IV antibiotics
  • Comorbidities complicating management

ICU Admission Criteria

  • Respiratory failure requiring NIV or intubation
  • Massive hemoptysis
  • Sepsis or hemodynamic instability
  • Hypercapnia with pH <7.25

Discharge Criteria

  • Stable or improving symptoms
  • Oxygen at or near baseline
  • Able to take oral medications
  • Airway clearance education provided
  • Follow-up arranged
  • Exacerbation action plan in place

Follow-up Recommendations

TimeframePurpose
2-4 weeksPost-exacerbation pulmonology review
Sputum cultureDuring exacerbation for future guidance
AnnualPulmonary function tests, CT if indicated

Patient Education

Understanding Bronchiectasis

  • Bronchiectasis is a chronic lung condition with widened airways
  • Airways become damaged and prone to repeated infections
  • Daily airway clearance is essential
  • Complete antibiotic courses as prescribed

Daily Management

Airway Clearance

  • Perform twice daily (more during exacerbations)
  • Techniques include huff coughing, active cycle breathing
  • Use hypertonic saline nebulizer if prescribed
  • Devices like flutter valves or vest therapy may help

Hydration

  • Good hydration helps thin secretions
  • Aim for 2-3L fluid daily unless restricted

Exacerbation Recognition

Seek care if:

  • Increased sputum volume or color change
  • Increased breathlessness
  • New or increased fever
  • Worsening fatigue
  • Blood in sputum

Prevention

  • Annual influenza vaccination
  • Pneumococcal vaccination
  • COVID-19 vaccination
  • Avoid respiratory irritants
  • Good hand hygiene
  • Treat GERD if present

Special Populations

Cystic Fibrosis-Related Bronchiectasis

  • Earlier onset, more severe
  • Pseudomonas colonization very common
  • Pancreatic insufficiency, diabetes, sinusitis
  • CF transmembrane conductance regulator (CFTR) modulators
  • Dornase alfa (Pulmozyme) effective for sputum

Immunodeficiency-Associated

  • Screen for immunoglobulin deficiency
  • IgG replacement therapy if deficient
  • More aggressive infection management

Non-Tuberculous Mycobacterial Infection

Suspect if:

  • Not responding to standard antibiotics
  • Nodular or cavitary disease
  • Low BMI, older female
  • MAC is most common

Management:

  • Multiple drug regimen (macrolide + rifampin + ethambutol)
  • Prolonged treatment (12+ months after culture negative)
  • Specialist involvement essential

ABPA (Allergic Bronchopulmonary Aspergillosis)

  • Sensitization to Aspergillus in airway
  • Eosinophilia, elevated IgE
  • Central bronchiectasis characteristic
  • Treatment: Oral corticosteroids, itraconazole

Quality Metrics

Performance Indicators

MetricTarget
Sputum culture sent during exacerbation>0%
Antibiotic selection based on prior cultures>0%
Oxygen saturation documented100%
Airway clearance education provided100%
Follow-up arranged100%
Vaccination status reviewed100%

Documentation Requirements

  • Baseline pulmonary status (FEV1, usual SpO2)
  • Prior cultures and sensitivities
  • Current symptoms vs baseline
  • Antibiotic choice rationale
  • Airway clearance plan
  • Discharge instructions

Key Clinical Pearls

Diagnostic Pearls

  1. CT chest is gold standard - don't rely on CXR alone
  2. Know the patient's baseline - colonization vs acute infection
  3. Always send sputum during exacerbations
  4. Consider NTM if not responding to standard therapy
  5. Screen for underlying causes - immunodeficiency, CF, ABPA

Treatment Pearls

  1. Pseudomonas requires specific coverage - ciprofloxacin or IV antipseudomonal
  2. 14 days of antibiotics is standard for exacerbations
  3. Airway clearance is as important as antibiotics
  4. Avoid over-oxygenation in chronic CO2 retainers
  5. Tranexamic acid is first-line for hemoptysis

Disposition Pearls

  1. Low threshold for admission in severe exacerbation
  2. Ensure baseline is documented for future reference
  3. Follow-up is essential - chronic disease requires continuity
  4. Exacerbation action plan empowers patients
  5. Pulmonology referral for new diagnosis or frequent exacerbations

References
  1. Polverino E, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017;50(3):1700629.
  2. Hill AT, et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019;74(Suppl 1):1-69.
  3. Martinez-Garcia MA, et al. Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score. Eur Respir J. 2014;43(5):1357-1367.
  4. Chalmers JD, et al. The bronchiectasis severity index. Am J Respir Crit Care Med. 2014;189(5):576-585.
  5. King PT. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4:411-419.
  6. Pasteur MC, et al. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65 Suppl 1:i1-58.

Version History
VersionDateChanges
1.02025-01-15Initial comprehensive version with 14-section template

At a Glance

EvidenceStandard
Last UpdatedRecently

Clinical Pearls

  • adjacent artery (signet ring) |

Guidelines

  • NICE Guidelines
  • BTS Guidelines
  • RCUK Guidelines