Bronchiectasis – MD Nexus

Definition

Bronchiectasis is Defined as the Permanent Dilation of Bronchi and Bronchioles with Associated Airway Wall Damage

Epidemiology

Prevalence

Bronchiectasis is Found in Patients of All Ages, Genders, and Ethnic Groups
- However, Older Adults are More Commonly Affected than Younger Age Groups

Sex

Females are More Commonly Affected than Males in North America

Association with Chronic Obstructive Pulmonary Disease (COPD) (see Chronic Obstructive Pulmonary Disease)

Patients with Moderate-Severe COPD Have an Increased Risk of Bronchiectasis (Chest, 2011) [MEDLINE]
These Patients Have Severe Airflow Obstruction, Isolation of a Pathogenic Organism from Sputum, and at Least One Hospital Admission for an Exacerbation in the Prior Year
These Patients Also Have an Increased Mortality Rate (Hazard Ratio: 2.54) (Am J Respir Crit Care Med, 2013) [MEDLINE]

Etiology

General Comments

Relative Prevalence of Specific Etiologies

Post-Infectious Bronchiectasis Cases are Uncommon in the United States (Except in Native Americans in Alaska), But are Common in Underdeveloped Countries
Approximately 25-30% of Bronchiectasis Cases Can Be Traced to a Prior Pulmonary Infection

Idiopathic

Over 50% of All Bronchiectasis Cases are Idiopathic

Immunodeficiency

Hypogammaglobulinemia (see Hypogammaglobulinemia)

Agammaglobulinemia (see Agammaglobulinemia)
- Autosomal Recessive Agammaglobulinemia: severe hypogammaglobulinemia, antibody deficiency, and increased susceptibility to infection
- X-Linked Agammaglobulinemia (Bruton Agammaglobulinemia): severe hypogammaglobulinemia, antibody deficiency, and increased susceptibility to infection
  - Most cases do not present prior to 6 mo of age
Ataxia-Telangiectasia
B-Cell Lymphoma (see Lymphoma): due to increased catabolism of immunoglobulins
Biallelic Deficiency of Mismatch Repair Protein PMS2
Common Variable Immunodeficiency (CVID) (see Common Variable Immunodeficiency): most cases do not present until the third or fourth decade of life
Drug-Related Hypogammaglobulinemia: these cases probably occur in specific predisposed individuals
- Captopril (see Captopril): may cause IgA deficiency
- Carbamazepine (Tegretol) (see Carbamazepine): may cause IgA deficiency or reversible hypogammaglobulinemia
- Chlorpromazine (Thorazine) (see Chlorpromazine): may cause IgA deficiency
- Hydroxychloroquine (Plaquenil) (see Hydroxychloroquine): may cause IgA deficiency
- Lamotrigine (Lamictal) (see Lamotrigine): may cause reversible hypogammaglobulinemia
- Penicillamine (see Penicillamine): may cause IgA deficiency
- Phenytoin (Dilantin) (see Phenytoin): may cause IgA deficiency or reversible hypogammaglobulinemia
- Ramipril (Altace) (see Ramipril): may cause hypogammaglobulinemia
- Sulfasalazine (see Sulfasalazine): may cause IgA deficiency
- Valproic Acid (Depakote, Depakene) (see Valproic Acid): may cause IgA deficiency
Good Syndrome (Immunodeficiency with Thymoma) (see Thymoma)
- Physiology
  - B-Cell Immunodeficiency Syndrome
- Clinical
  - Hypogammaglobulinemia (see Hypogammaglobulinemia)
  - Thymoma (see Thymoma): usually spindle cell histology
Hyper-IgM Syndrome
- CD40 Ligand (CD40L or CD154) Deficiency: this X-linked disorder is the etiology of most cases of Hyper-IgM syndrome
- CD40 Deficiency
- Activation-Induced Cytidine Deaminase (AID) Deficiency
- Uracil-Nucleoside-Glycosylase (UNG) Deficiency
Immunodeficiency-Centromeric Instability-Facial Anomalies (ICF) Syndrome
- Normal B-cell counts with agammaglobulinemia
Jacobsen Syndrome (Hemizygous Deletion of Part of the Long Arm of Chromosome 11)
Immunosuppression
- Autologous Hematopoietic Stem Cell (HSCT) (Bone Marrow Transplant, BMT) (see Hematopoietic Stem Cell Transplant)
- Azathioprine (Imuran) (see Azathioprine)
- Belimumab (Benlysta) (See Belimumab): anti-B cell monoclonal antibody
- Chemotherapy
- Corticosteroids (see Corticosteroids)
  - Patients taking ≥12.5 mg prednisone for at least 1 year are at increased risk of hypogammaglobulinemia
  - Patients with hypogammaglobulinemia due to corticosteroid usually retain specific antibody responses: therefore, they are not usually candidates for immunoglobulin replacement therapy
- Gold (see Gold)
- Mycophenolate Mofetil (Cellcept) (see Mycophenolate Mofetil): may cause hypogammaglobulinemia
- Rituximab (Rituxan) (see Rituximab)
- Tyrosine Kinase Inhibitors
  - Dasatinib (Sprycel) (see Dasatinib)
  - Imatinib (Gleevec) (see Imatinib)
Isolated IgA Deficiency (see Isolated IgA Deficiency): few reported cases of bronchiectasis (although these were probably due to undetected IgG deficiencies): most common primary immunodeficiency syndrome (occurs in 1:600 persons)
Lymphoproliferative Malignancy
- Chronic Lymphocytic Leukemia (CLL) (see Chronic Lymphocytic Leukemia): commonly associated with hypogammaglobulinemia and infection
- Multiple Myeloma (see Multiple Myeloma): antibody deficiency with normal total IgG levels (due to contribution of the paraprotein to the total IgG level and due to tumor cells altering normal regulatory T cells, impairing B-cell maturation)
Myelodysplastic Syndrome (MDS) (see Myelodysplastic Syndrome)
Myotonic Dystrophy
- Increased catabolism of immunoglobulins
Nephrotic Syndrome (see Nephrotic Syndrome)
- Diagnosis
  - Hypoalbuminemia (see Hypoalbuminemia)
- Clinical
  - Edema: usually
Premature Birth
- Premature infants delivered before the third trimester usually lack adequate maternal immunoglobulin and may also more rapid metabolize the IgG that they have received
Protein-Losing Enteropathy
- XXX
  - Intestinal Lymphangiectasia
- Diagnosis
  - IgG levels are typically affected more than IgM or IgA levels (however, the levels of IgG, IgM, and IgA may all be decreased in severe protein-losing enteropathy)
  - Hypoalbuminemia (see Hypoalbuminemia)
- Clinical
  - Edema: usually
Selective IgG Subclass Deficiency (see Selective IgG Subclass Deficiency): decrease in one or more of the four classes of IgG with normal total IgG is most common type associated with bronchiectasis
Severe Burns (see Burns)
- Physiology
  - Increased Catabolism of Immunoglobulins
Severe Combined Immunodeficiency (SCID) (see Severe Combined Immunodeficiency)
- Epidemiology: xxx
Specific Antibody Deficiency (SAD)/Specific Polysaccharide Antibody Deficiency (SPAD) (see Specific Antibody Deficiency): poor serological response to polysaccharide antigens (with normal levels of immunoglobulins and IgG subclasses) and normal responses to protein antigens
Steinert’s Disease
Transcobalamine Deficiency
Trisomy 18
Wiskott-Aldrich Syndrome

Other

Chronic Granulomatous Disease (CGD) (see Chronic Granulomatous Disease)

Infection

Adenovirus-Type 7 (see Adenovirus)
Allergic Bronchopulmonary Aspergillosis (ABPA) (see Allergic Bronchopulmonary Aspergillosis)
- Clinical
  - Allergic Bronchopulmonary Aspergillosis Typically Produces Upper Lobe Bronchiectasis (Mainly Apical and Posterior Segments)
Influenza Virus (see Influenza Virus)
- Physiology
  - Studies Have Shown Transient Nasal Epithelial Ciliary Loss and Dynein Arm Abnormalities in Children During Viral Upper Respiratory Infection (Influenza, Parainfluenza, etc)
Measles Virus (see Measles Virus)
Mycobacterium Avium Complex (MAC) (see Mycobacterium Avium Complex)
Mycobacterium Tuberculosis (see Tuberculosis)
- Epidemiology
  - Bronchiectasis May Occur Following Primary Tuberculosis or Reactivation Tuberculosis
- Diagnosis
  - Tuberculosis Typically Produces Upper Lobe Bronchiectasis (mainly in the Apical and Posterior Segments)
Necrotizing Pneumonia and Pulmonary Gangrene (see Necrotizing Pneumonia and Pulmonary Gangrene)
Pertussis (see Pertussis)
Recurrent Aspiration Pneumonia (see Aspiration Pneumonia)
- Physiology
  - Bronchiectasis in Association with Recurrent Aspiration Pneumonia Probably Results from Combined Injury from Infection and Gastric Acid

Inhalation Injury

Ammonia Inhalation (see Ammonia)
Sulfur Mustard Gas Inhalation (see Sulfur Mustard Gas)
Smoke Inhalation (see Smoke Inhalation)
Recurrent Aspiration Pneumonia (see Aspiration Pneumonia)
- Physiology
  - Bronchiectasis in Association with Recurrent Aspiration Pneumonia Probably Results from Combined Injury from Infection and Gastric Acid

Other

Alpha-1 Antitrypsin Deficiency (see Alpha-1 Antitrypsin Deficiency)
Chronic Lung Transplant Rejection (see Lung Transplant)
Congenital Bronchiectasis
Cystic Fibrosis (CF) (see Cystic Fibrosis)
Ehlers-Danlos Syndrome (see Ehlers-Danlos Syndrome)
Inflammatory Bowel Disease (IBD) (see Inflammatory Bowel Disease)
- Clinical
  - The Large Airways are the Most Common Site of Lung Involvement in Inflammatory Bowel Disease
    - Bronchiectasis is the Most Common Manifestation
  - Some Cases (Predominantly Ulcerative Colitis) Have Recurrence of Bronchiectasis After Colectomy
Localized Airway Obstruction
- Airway Foreign Body (see Airway Foreign Body)
- Endobronchial Tumors: bronchial adenoma, bronchial carcinoid, lung cancer, endrobronchial metastases, etc
- Middle Lobe Syndrome (see Middle Lobe Syndrome)
Marfan’s Syndrome (see Marfan Syndrome)
Mounier-Kuhn Syndrome (see Mounier-Kuhn Syndrome)
- Clinical
  - Tracheobronchomegaly
Peripheral Neuropathy (see Peripheral Neuropathy)
- Epidemiology
  - Two Reported Cases Had Resolution of Neuropathy with Treatment of Bronchiectasis
Primary Ciliary Dyskinesia (see Primary Ciliary Dyskinesia)
Pulmonary Sequestration (see Pulmonary Sequestration)
Rheumatoid Arthritis (RA) (see Rheumatoid Arthritis)
Sarcoidosis (see Sarcoidosis)
Scleroderma (see Scleroderma)
- Epidemiology
  - Cylindrical Bronchiectasis is Common in Scleroderma
- Physiology
  - Due to Recurrent Aspiration or Traction Bronchiectasis Associated with Interstitial Lung Disease
Swyer-James-Macleod Syndrome (see Swyer-James-Macleod Syndrome)
Unilateral Pulmonary Artery Agenesis (see Unilateral Pulmonary Artery Agenesis)
Williams-Campbell Syndrome (see Williams-Campbell Syndrome)
- Physiology
  - Congenital Cartilage Deficiency
Yellow Nail Syndrome (see Yellow Nail Syndrome)

Physiology

Bronchiectasis Involves the Destruction of Muscular and Elastic Components of Medium-Sized Bronchial Walls by Mediators Released by Neutrophils (Elastase) and Monocytes (Cytokines), Resulting in >2 mm Dilatation of Medium-Sized Bronchi

Damage to Peribronchial Alveolar Tissue
- Diffuse Peribronchial Fibrosis, Squamous Metaplasia of Bronchial Epithelium, and Obliteration of Distal Bronchi and Bronchioles
Impaired Tracheobronchial Clearance of Secretions (Demonstrated by Radiolabelled Aerosol Studies)
- Predisposes to Bacterial Airway Colonization and Infection

Specific Role of Infection in the Pathogenesis of Bronchiectasis

It is Unclear Whether Mycobacterial Infection is a Cause or a Consequence of Bronchiectasis (Clin Chest Med, 2012) [MEDLINE]
- Pseudomonas Aeruginosa Infection Appears to Be Associated with More Severe Disease and Cystic Bronchiectasis (see Pseudomonas Aeruginosa)
- Mycobacterium Avium Complex (MAC) Infection Appears to Be Associated with Nodular Bronchiectasis (Especially in the Right Middle Lobe and Lingula), Mucous Plugging of Airways, and “Tree-in-Bud” Small Airway Impaction (see Mycobacterium Avium Complex)

Reversibility of Bronchiectasis

Bronchiectasis May Resolve After Even Years of Observation in Some Cases (Especially in Areas of Atelectasis Due to Previous Pneumonia), Although Generally is Believed to Be Permanent

Location of Bronchiectasis

Bronchiectasis Can Be Diffuse or Localized
Bronchiectasis Most Commonly Involves the Posterior-Basal Segments
Lower Lobe Involvement
- Bilateral Lower Lobe Involvement Occurs in 33% of Cases
- Unilateral Lower Lobe Involvement Occurs in Left and Right Lungs with Equal Frequency
- Approximately 50% of Patients with Left Lower Lobe Involvement Also Have lingular involvement
Right Middle Lobe Involvement
- Right Middle Lobe is More Commonly Affected than the Right Upper Lobe
Upper Lobe Involvement
- Upper Lobe Involvement Most Common in Posterior and Apical Segments (and is Usually Due to Allergic Bronchopulmonary Aspergillosis and Tuberculosis) (see Allergic Bronchopulmonary Aspergillosis and Tuberculosis)

Pathologic Classification (Reid)

General Comments

There is No Epidemiologic or Etiologic Significance to This Classification

Classification

Cylindrical: consistent widening of segments
Varicose: local constrictions in cylindrical segments (resembling varicose veins)
Saccular/Cystic: dilatation increases toward lung periphery (forming sacs)

Diagnosis

Arterial Blood Gas (ABG) (see Arterial Blood Gas)

Findings

Mild Hypoxemia with Normocapnia/Hypocapnia (see Hypoxemia and Hypocapnia): hypoxemia is due to intrapulmonary shunt and V/Q mismatch
Hypercapnia (see Hypercapnia): uncommon and is seen only in advanced cases or in those patients with superimposed COPD

Sputum Gram Stain and Culture (see Sputum Culture)

Classic 3-Layered Sputum

Top Layer: frothy watery layer
Middle Layer: turbid mucopurulent layer
Bottom Layer: purulent opaque layer (bottom layer may also contain Dittrich’s plugs and/or yellow or white concretions)

Sputum Culture

Pseudomonas Aeruginosa (see Pseudomonas Aeruginosa)
- Colonizes Bronchiectasis Patients in 33% of Cases
Haemophilus Influenzae (see Haemophilus Influenzae)
- Common
Other Gram-Negatve Rods
- Common
Staphylococcus Aureus (see Staphylococcus Aureus)
- May Be Seen in Some Cases
Streptococcus Pneumoniae (see Streptococcus Pneumoniae)
- May Be Seen in Some Cases
Non-Tuberculous Mycobacteria
Commonly Cultured in North American Patients
Mycobacterium Avium Complex (MAC) (see Mycobacterium Avium Complex)
- Most Common Mycobacteria Found in Bronchiectasis Patients
Mycobacterium Abscessus (see Mycobacterium Abscessus)

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests)

General Comments

Most Bronchiectasis Studies are Flawed, Since Concurrent Chronic Obstructive Pulmonary Disease (COPD) Cannot Be Excluded
- However, Many Cases of Bronchiectasis Manifest Airway Obstruction by Pulmonary Function Tests

Findings

FEV1: usually decreased
FEF25-75: may be decreased
Mild Restriction: may be seen in some cases (especially if underlying disease decreases volumes)
DLCO: may be decreased

Bronchoscopy (see Bronchoscopy)

General Comments

Bronchoscopy is Useful to Evaluate for Sources of Hemoptysis, Rule Out Foreign Bodies, etc
- Transbronchial Biopsy (TBB) is Generally Not Indicated

Findings

Airways May Appear Wider than Normal on Visual Examination

Chest X-Ray (CXR) (see Chest X-Ray)

General Comments

Chest X-Ray is Commonly Used as the First Imaging Procedure to Evaluate for the Presence of Bronchiectasis
- However, the Sensitivity of Chest X-Ray is Low for Bronchiectasis, as Compared to CT Scan

Findings

Cylindrical Bronchiectasis: tram-tracks
Varicose Bronchiectasis: toothpaste lines
Cystic Bronchiectasis: cysts that may contain fluid

Chest Computed Tomography (Chest CT) (see Chest Computed Tomography)

General Comments

Non-Contrast Chest Computed Tomography is Commonly Used as the Second Procedure to Diagnose the Presence of Bronchiectasis (and is Typically Adequate, Often Obviating the Need for High-Resolution Chest CT)

Findings

Cylindrical Bronchiectasis: tram-tracks
Varicose Bronchiectasis: toothpaste lines
Cystic Bronchiectasis: cysts that may contain fluid

High-Resolution Chest Computed Tomography (HRCT) (see High-Resolution Chest CT)

General Comments

High-Resolution Chest Computed Tomography (HRCT) is Considered the Gold Standard Imaging Modality

Performance

Sensitivity: 84%
Specificity: 82%

Findings

Bronchial Wall Thickening and Dilatation

Bronchography

General Comments

Bronchography is of Historical Interest Only and is No Longer Used

Serum Alpha-1 Antitrypsin (A1AT) (see Serum Alpha-1 Antitrypsin)

General Comments

Serum Alpha-1 Antitrypsin Level Measurement is Useful to Rule Out Alpha-1 Antitrypsin Deficiency (see Alpha-1 Antitrypsin Deficiency)

Immunoglobulin Levels (see xxxx)

General Comments

Immunoglobulin Levels are Useful to Rule Out Underlying Immunodeficiency

Sweat Chloride Test (see Sweat Chloride Test)

General Comments

Sweat Chloride Test is Useful to Rule Out Cystic Fibrosis (CF) (see Cystic Fibrosis)

Electron Microscopic Exam of Sperm or Respiratory Epithelium (Via Nasal Biopsy)

General Comments

Electron Microscopic Exam of Sperm or Respiratory Epithelium is Useful to Rule Out Ciliary Disorder

Clinical Manifestations

Cardiovascular Manifestations

Cardiovascular Events (Arrhythmias, Ischemia, Congestive Heart Failure, etc)

Epidemiology
- By Population Studies, There is an Increased Risk of Cardiovascular Events Following a Bronchiectasis Exacerbation (Chest, 2021) [MEDLINE]
  - During a Median Follow-Up of 35 mos, 29.6% of Patients Had a Cardiovascular Events and 37.2% Died
  - Semi-Competing Risk Analysis Indicated that Age, Arterial Hypertension, Chronic Obstructive Pulmonary Disease (COPD), and Potentially Severe Exacerbations Significantly Increased the Risk for Developing Cardiovascular Events
  - Compared with Patients without Cardiovascular Events, Those with Cardiovascular Events Had a Higher Mortality Rate

Otolaryngologic Manifestations

Recurrent/Chronic Rhinosinusitis

Epidemiology
- Nasal/Sinus Disease May Be Seen in Association with Bronchiectasis Related to B-Cell Dysfunction Disorders

Pulmonary Manifestations

Chronic Hypoxemic, Hypercapnic Respiratory Failure (see Respiratory Failure)

Epidemiology
- Chronic Hypoventilation (Hypoxemic, Hypercapnic Respiratory Failure) Typically Occurs Only in Patients with Advanced Bronchiectasis or in Those with Superimposed Chronic Obstructive Pulmonary Disease (COPD) (see Chronic Obstructive Pulmonary Disease)

Cough with/without Sputum Production (see Cough): most common symptom

Clinical
- “Wet Bronchiectasis”: purulent foul-smelling sputum
  - Mild: <10 mL per day
  - Moderate: 10-150 mL
  - Severe: >150 mL
- “Dry Bronchiectasis”: some cases (especially those involving upper lobes that dependently drain) have minimal sputum

Dyspnea (see Dyspnea)

Epidemiology
- Dyspnea is More Common in Patients with Extensive Bronchiectasis and in Those with Superimposed Chronic Obstructive Pulmonary Disease (COPD) (see Chronic Obstructive Pulmonary Disease)

Empyema (see Pleural Effusion-Parapneumonic)

Epidemiology
- XXXXXXXX

Hemoptysis (see Hemoptysis)

Epidemiology
- Hemoptysis is More Common in Dry Bronchiectasis
Clinical
- Bronchiectasis-Associated Hemoptysis is Usually Mild, But May Be Massive (>250 mL/Day)
- Bronchiectasis-Associated Hemoptysis is Rarely Fatal

Lung Abcess (see Lung Abscess)

Epidemiology
- XXXXX

Pneumothorax (see Pneumothorax)

Epidemiology
- XXXXX

Pulmonary Hypertension/Cor Pulmonale (see Pulmonary Hypertension)

Epidemiology
- Pulmonary Hypertension Seen in Some Cases (37% of Cases by 1969 Study)
Physiology
- Enlargement of Bronchial Arteries: due to increased flow to tissue
- Anastomoses to Pulmonary Arteries: due to enlargement of preexisting connections and new connections
- Total Pulmonary Arterial Flow: may be decreased in severe bronchiectasis, due to obstructive endarteritis and/or hypoxic vasoconstriction
- Left-to-Right Intrapulmonary Shunting Occurs: due to anastomoses

Rales/Rhonchi (see Pulmonary Physical Exam)

Epidemiology
- XXXX

Recurrent Pneumonia (see Community-Acquired Pneumonia)

Epidemiology
- XXXX

Wheezing (see Wheezing)

Epidemiology
- XXXX

Rheumatologic Manifestations

Clubbing (see Clubbing)

Physiology
- Correlation Between Augmented Vascular Supply in Lung and Vascularity in Clubbed Digits of Bronchiectasis Patients (Suggests a Possible Vasodilator Substance Which Has Not Been Yet Identified)

Other Manifestations

Amyloidosis (see Amyloidosis)

Epidemiology
- Bronchiectasis-Associated Amyloidosis Rarely Seen Today in Developed Countries

Brain Abscess (see Brain Abscess)

Epidemiology
- XXXX

Fever (see Fever)

Epidemiology
- XXXX

Generalized Weakness

Epidemiology
- XXXX

Weight Loss (see Weight Loss)

Epidemiology
- XXXX

Prevention

Vaccination

Measles Vaccination (see Measles Virus)
Pertussis Vaccination (see Pertussis)
Influenza Vaccination (see Influenza Virus)

Prompt Removal of Foreign Bodies in Airway

xxxx

Treatment

Treatment of Hemoptysis

Bronchoscopy (see Bronchoscopy): to localize (may tamponade with Fogarty balloon)
Bronchial Artery (and Sometimes Also Pulmonary Artery) Embolization: may be required
- Complications: spinal artery occlusion with spinal cord infarction (see Spinal Cord Infarction)
Surgery: may be required in some cases for refractory hemoptysis

Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin)

Prevents Exacerbations in the Setting of Immunoglobulin Deficiency

Postural Drainage

xxx

Chest Physical Therapy (PT) (see Chest Physical Therapy)

xxx

Humidification

xxx

Surgery

Indications: localized bronchiectasis with recurrent infection unresponsive to antibiotics

Mucolytics

Agents

Coughing is Probably Superior

Agents

Deoxyribonuclease (DNase) (see Dornase Alfa): useful only for cystic fibrosis, as trials suggest that it has potential harmful effects in non-cystic bronchiectasis (Cochrane Database Syst Rev, 2014) [MEDLINE]
Bromhexine with Antibiotics: may facilitate sputum production and clearance, but long-term data and clinical outcomes are lacking
Erdosteine: may be a useful adjunct to physical therapy in stable patients with mucus hypersecretion, but robust longer-term trials are required
N-Acetylcysteine (Mucomyst) (see N-Acetylcysteine): further studies are required

Clinical Efficacy

xxxxx

Antibiotics

Clinical Utility

Antibiotic Treatment of Acute Exacerbation is Standard Therapy
Antibiotic Maintenance Therapy is Indicated for Patients with >2-3 Exacerbations Per Year

Fluoroquinolones (see Fluoroquinolones)

Agents
- Ciprofloxacin (Cipro) (see Ciprofloxacin)
- Levofloxacin (Levaquin) (see Levofloxacin)
- Ofloxacin (see Ofloxacin)
Clinical Efficacy
- During course of therapy, fluoroquinolones decrease sputum elastase, neutrophil chemotactic activity, sputum volume and purulence: however, 25% of patients relapse within 6 wks after therapy

Gentamicin (Nebulized) (see Gentamicin)

Clinical Efficacy
- Randomized Trial of Nebulized Gentamicin in Non-Cystic Bronchiectasis (Am J Respir Crit Care Med, 2011 ) [MEDLINE]
  - Regular long-term nebulized gentamicin 80 mg BID decreased sputum bacterial density, airway inflammation, and exacerbations (with no change in FEV1 or FVC)
  - Treatment needs to be continuous for ongoing efficacy
- Systematic Review and Meta-Analysis of Inhaled Antibiotics in Bronchiectasis in Adults (Lancet Respir Med, 2019) [MEDLINE]: n = 2,597 (16 trials)
  - Inhaled antibiotics are well tolerated, reduce bacterial load, and achieve a small but statistically significant reduction in exacerbation frequency without clinically significant improvements in quality of life in patients with bronchiectasis and chronic respiratory tract infections

Tobramycin (Tobi) (see Tobramycin)

Clinical Efficacy
- Systematic Review and Meta-Analysis of Inhaled Antibiotics in Bronchiectasis in Adults (Lancet Respir Med, 2019) [MEDLINE]: n = 2,597 (16 trials)
  - Inhaled Antibiotics are Well-Tolerated, Reduce Bacterial Load, and Achieve a Small But Statistically Significant Reduction in Exacerbation Frequency without Clinically Significant Improvements in Quality of Life in Patients with Bronchiectasis and Chronic Respiratory Tract Infections

Macrolides (see Macrolides)

Agents
- Azithromycin (see Azithromycin)
- Clarithromycin (see Clarithromycin)
- Erythromycin (see Erythromycin)
Clinical Efficacy
- EMBRACE Trial (Lancet, 2012) [MEDLINE]
  - Azithromycin x 6 mo Decreased Rate of Exacerbations (in Patients Who Had at Least One Exacerbation in the Past Year)
- BAT Trial (JAMA, 2013) [MEDLINE]: multi-center Dutch placebo-controlled study (n = 83) in non-cystic fibrosis bronchiectasis using daily azithromycin x 12 mo
  - Decreased Rate of Infectious Exacerbations
  - Increased Rate of Macrolide Resistance
- BLESS Trial (JAMA, 2013) [MEDLINE]: single-center Australian placebo-controlled study (n = 117) in non-cystic fibrosis bronchiectasis using daily erythromycin x 12 mo
  - Modest Decrease in the Rate of Exacerbations
  - Increased Rate of Macrolide Resistance
  - Decreased Sputum Production
- Analysis of BLESS Trial Data (Lancet Respir Med, 2014) [MEDLINE]
  - Long-Term Erythromycin Treatment Changes the Composition of Respiratory Microbiota in Bronchiectasis Patients
  - In Patients without Pseudomonas Aeruginosa Airway Infection, Erythromycin Did Not Significantly Decrease Exacerbations and Promoted Displacement of Haemophilus Influenzae by More Macrolide-Tolerant Pathogens (Including Pseudomonas Aeruginosa)
    - These Findings Argue for a Cautious Approach to Chronic Macrolide Use in Patients without Pseudomonas Aeruginosa Airway Infection
- Meta-Analysis of Long-Term Macrolides in Bronchiectasis in Adults (Lancet Resp Med, 2019) [MEDLINE]: n = 234 studies with low risk of bias (included 3 randomized, controlled trials)
  - Long-term Macrolide Treatment Significantly Reduced the Frequency of Bronchiectasis Exacerbations (Adjusted Incidence Rate Ratio 0.49; 95% CI: 0.36-0.66; p < 0.0001)
    - Effect Estimates in Prespecified Subgroup Analyses Revealed a Decreased Frequency of Exacerbations in All Prespecified Subgroups, Including a High Level of Benefit in Patients with Pseudomonas Aeruginosa Infection (Incidence Rate Ratio 0.36; 95% CI: 0.18-0.72; p = 0.0044) and in Patients with 1-2 Exacerbations Per Year (0.37; 95% CI: 0.16-0.88; p = 0.025)
  - Long-term Macrolide Treatment Improved the Time to First Exacerbation (Adjusted Hazard Ratio 0.46; 95% CI: 0.34-0.61; p < 0.0001)
  - Long-term Macrolide Treatment was Associated with Improved Quality of Life, as Measured by the St. George’s Respiratory Questionnaire (SGRQ) (Mean Improvement 2.93 Points; 95% CI: 0.03-5.83; p = 0.048)
  - Long-term Macrolide Treatment was Not Associated with a Significant Improvement in FEV1 (67 mL at 1 Year; 95% CI: -22 to 112; p = 0.14)
  - However, the Potential Downsides of Long-Term Macrolide Treatment Must Be Considered

Penicillins (see Penicillins)

Agents
- Amoxacillin (see Amoxacillin)

Tetracyclines (see Tetracyclines)

Agents
- Doxycycline (see Doxycycline)
- Tetracycline (see Tetracycline)

Sulfamethoxazole-Trimethoprim (Bactrim, Septra) (see Sulfamethoxazole-Trimethoprim)

Clinical Efficacy
- XXXXX

Other Treatments

Nutrition

Nutritional Support is Indicated to Reverse Weight Loss

Inhaled Indomethacin (see Indomethacin)

Decreases Bronchorrhea (Anti-Inflammatory)

Bronchodilators

Terbutaline Increases Tracheal Mucous Velocity in CF Patients (see Terbutaline)

Oxygen Therapy (see Oxygen)

Oxygen Therapy is Probably Useful (if Required)

Prognosis

Prognosis of Bronchiectasis was Previously Poor in the Pre-Antibiotic Era

However, the Current Prognosis is Comparable to that of Chronic Obstructive Pulmonary Disease (COPD) (see Chronic Obstructive Pulmonary Disease)
- Functional Status: 77% of patients miss <2 wks of work annually

References

General

Saliva immunoglobulins in elite women rowers. Eur Resp J 15:5-10, 2000 [MEDLINE]
Thoracic manifestations of inflammatory bowel disease. Chest 131:524-532, 2007 [MEDLINE]
British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010 Jul;65 Suppl 1:i1-58 [MEDLINE]
Factors Associated With Bronchiectasis in Patients With COPD. Chest. 2011 Nov;140(5):1130-7 [MEDLINE]
The initial evaluation of adults with bronchiectasis. Clin Chest Med. 2012 Jun;33(2):219-31 [MEDLINE]
Bronchiectasis and nontuberculous mycobacterial disease. Clin Chest Med. 2012 Jun;33(2):283-95 [MEDLINE]
Prognostic Value of Bronchiectasis in Patients with Moderate-to-Severe Chronic Obstructive Pulmonary Disease. Am J Respir Crit Care Med. 2013 Apr 15;187(8):823-31 [MEDLINE]

Clinical

Cardiovascular Events During and After Bronchiectasis Exacerbations and Long-term Mortality. Chest. 2021 Oct 15;S0012-3692(21)04101-5. doi: 10.1016/j.chest.2021.10.013 [MEDLINE]

Treatment

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The effect of long-term macrolide treatment on respiratory microbiota composition in non-cystic fibrosis bronchiectasis: an analysis from the randomised, double-blind, placebo-controlled BLESS trial. Lancet Respir Med. 2014 Dec;2(12):988-96. doi: 10.1016/S2213-2600(14)70213-9. Epub 2014 Oct 14 [MEDLINE]
Mucolytics for bronchiectasis. Cochrane Database Syst Rev. 2014 May 2;5:CD001289. doi: 10.1002/14651858.CD001289.pub2 [MEDLINE]
Long-term macrolide antibiotics for the treatment of bronchiectasis in adults: an individual participant data meta-analysis. Lancet Respir Med. 2019 Oct;7(10):845-854. doi: 10.1016/S2213-2600(19)30191-2 [MEDLINE]
The efficacy and safety of inhaled antibiotics for the treatment of bronchiectasis in adults: a systematic review and meta-analysis. Lancet Respir Med. 2019 Oct;7(10):855-869. doi: 10.1016/S2213-2600(19)30185-7 [MEDLINE]