Bronchiectasis

Epidemiology

  • Definition: permanent dilation of bronchi and bronchioles with associated airway wall damage
  • Prevalence: bronchiectasis is found in patients of all ages, genders, and ethnic groups
    • However, older adults are mre commonly affected than youngr age groups
  • Sex: females are more commonly affected than males in North America
  • Relative Prevalence of Specific Etiologies
    • Post-infectious cases are uncommon in USA (except in Native Americans in Alaska) but are common in underdeveloped countries
    • Over 50% of bronchiectasis cases are idiopathic
    • Approximately 25-30% of bronchiectasis cases can be traced to a prior pulmonary infection
  • Association with COPD: patients with moderate-severe COPD have increased risk of bronchiectasis [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) [MEDLINE]

Etiology

Idiopathic

  • Over 50% of all bronchiectasis cases are idiopathic

Immunodeficiency

Hypogammaglobulinemia (see Hypogammaglobulinemia, [[Hypogammaglobulinemia]])

  • Ataxia-Telangiectasia
  • Autosomal Recessive Agammaglobulinemia (ARA)
  • B-Cell Lymphoma (see Lymphoma, [[Lymphoma]]): due to increased catabolism of immunoglobulins
  • Biallelic Deficiency of Mismatch Repair Protein PMS2
  • Bruton Agammaglobulinemia (X-Linked Aggamglobulinemia) (see Bruton Agammaglobulinemia, [[Bruton Agammaglobulinemia]]): most cases do not present prior to 6 mo of age
  • Common Variable Immunodeficiency (CVID) (see Common Variable Immunodeficiency, [[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, [[Captopril]]): may cause IgA deficiency
    • Carbamazepine (Tegretol) (see Carbamazepine, [[Carbamazepine]]): may cause IgA deficiency or reversible hypogammaglobulinemia
    • Chlorpromazine (Thorazine) (see Chlorpromazine, [[Chlorpromazine]]): may cause IgA deficiency
    • Hydroxychloroquine (Plaquenil) (see Hydroxychloroquine, [[Hydroxychloroquine]]): may cause IgA deficiency
    • Lamotrigine (Lamictal) (see Lamotrigine, [[Lamotrigine]]): may cause reversible hypogammaglobulinemia
    • Penicillamine (see Penicillamine, [[Penicillamine]]): may cause IgA deficiency
    • Phenytoin (Dilantin) (see Phenytoin, [[Phenytoin]]): may cause IgA deficiency or reversible hypogammaglobulinemia
    • Ramipril (Altace) (see Ramipril, [[Ramipril]]): may cause hypogammaglobulinemia
    • Sulfasalazine (see Sulfasalazine, [[Sulfasalazine]]): may cause IgA deficiency
    • Valproic Acid (Depakote, Depakene) (see Valproic Acid, [[Valproic Acid]]): may cause IgA deficiency
  • Good Syndrome (Immunodeficiency with Thymoma) (see Thymoma, [[Thymoma]])
  • 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 Bone Marrow Transplant (BMT)/Autologous Stem Cell Transplant (SCT) (see Bone Marrow Transplant, [[Bone Marrow Transplant]])
    • Azathioprine (Imuran) (see Azathioprine, [[Azathioprine]])
    • Belimumab (Benlysta) (See Belimumab, [[Belimumab]]): anti-B cell monoclonal antibody
    • Chemotherapy
    • Corticosteroids (see Corticosteroids, [[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, [[Gold]])
    • Mycophenolate Mofetil (Cellcept) (see Mycophenolate Mofetil, [[Mycophenolate Mofetil]]): may cause hypogammaglobulinemia
    • Rituximab (Rituxan) (see Anti-CD20 Therapy, [[Anti-CD20 Therapy]])
    • Tyrosine Kinase Inhibitors
      • Dasatinib (Sprycel) (see Dasatinib, [[Dasatinib]])
      • Imatinib (Gleevec) (see Imatinib, [[Imatinib]])
  • Isolated IgA Deficiency (see Isolated IgA Deficiency, [[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, [[Chronic Lymphocytic Leukemia]]): commonly associated with hypogammaglobulinemia and infection
    • Multiple Myeloma (see Multiple Myeloma, [[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 (see Myelodysplastic Syndrome, [[Myelodysplastic Syndrome]])
  • Myotonic Dystrophy: increased catabolism of immunoglobulins
  • Nephrotic Syndrome (see Nephrotic Syndrome, [[Nephrotic Syndrome]])
    • Hypoalbuminemia (see Hypoalbuminemia, [[Hypoalbuminemia]])
    • Edema: usually
  • Prematurity in Infants: 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: intestinal lymphangiectasia, etc
    • 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, [[Hypoalbuminemia]])
    • Edema: usually
  • Selective IgG Subclass Deficiency (see Selective IgG Subclass Deficiency, [[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, [[Burns]]): increased catabolism of immunoglobulins
  • Severe Combined Immunodeficiency (SCID)
  • Specific Antibody Deficiency (SAD)/Specific Polysaccharide Antibody Deficiency (SPAD) (see Specific Antibody Deficiency, [[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

Infection

  • Adenovirus-Type 7 (see Adenovirus, [[Adenovirus]])
  • Allergic Bronchopulmonary Aspergillosis (ABPA) (see Allergic Bronchopulmonary Aspergillosis, [[Allergic Bronchopulmonary Aspergillosis]])
    • Typically produces upper lobe bronchiectasis (mainly apical and posterior segments)
  • Influenza Virus (see Influenza Virus, [[Influenza Virus]])
    • Studies have shown transient nasal epithelial ciliary loss and dynein arm abnormalities in children during viral URI (influenza/ parainfluenza/ etc)
  • Measles Virus (see Measles Virus, [[Measles Virus]])
  • Mycobacterium Avium Complex (MAC) (see Mycobacterium Avium Complex, [[Mycobacterium Avium Complex]])
  • Mycobacterium Tuberculosis (see Tuberculosis, [[Tuberculosis]])
    • Typically produces upper lobe bronchiectasis (mainly apical and posterior segments)
  • Necrotizing Pneumonia and Pulmonary Gangrene (see Necrotizing Pneumonia and Pulmonary Gangrene, [[Necrotizing Pneumonia and Pulmonary Gangrene]])
  • Pertussis (see Pertussis, [[Pertussis]])
  • Recurrent Aspiration Pneumonia (see [[Aspiration Pneumonia]]): bronchiectasis probably results from combined injury from infection and gastric acid

Inhalation injury

  • Ammonia Inhalation (see Ammonia, [[Ammonia]])
  • Sulfur Mustard Gas Inhalation (see Sulfur Mustard Gas, [[Sulfur Mustard Gas]])
  • Smoke Inhalation (see Smoke Inhalation, [[Smoke Inhalation]])
  • Recurrent Aspiration Pneumonia (see Aspiration Pneumonia, [[Aspiration Pneumonia]]): bronchiectasis probably results from combined injury from infection and gastric acid

Other

  • Alpha-1 Antitrypsin Deficiency (see Alpha-1 Antitrypsin Deficiency, [[Alpha-1 Antitrypsin Deficiency]])
  • Chronic Lung Transplant Rejection (see Lung Transplant, [[Lung Transplant]])
  • Congenital Bronchiectasis
  • Cystic Fibrosis (CF) (see Cystic Fibrosis, [[Cystic Fibrosis]])
  • Ehlers-Danlos Syndrome (see Ehlers-Danlos Syndrome, [[Ehlers-Danlos Syndrome]])
  • Inflammatory Bowel Disease (see Inflammatory Bowel Disease, [[Inflammatory Bowel Disease]])
    • The large airways are the most common site of lung involvement in inflammatory bowel disease (affecting 52% of patients), with bronchiectasis being the most common manifestation
    • Some cases (mostly ulcerative colitis) have recurrence of bronchiectasis after colectomy
  • Localized Airway Obstruction
    • Foreign Body (see Foreign Body, [[Foreign Body]])
    • Middle Lobe Syndrome (see Middle Lobe Syndrome, [[Middle Lobe Syndrome]])
    • Endobronchial Tumors: bronchial adenoma, bronchial carcinoid, lung cancer, endrobronchial metastases, etc
  • Marfan’s Syndrome (see Marfan Syndrome, [[Marfan Syndrome]])
  • Mounier-Kuhn Syndrome (see Mounier-Kuhn Syndrome, [[Mounier-Kuhn Syndrome]]): tracheobronchomegaly
  • Peripheral Neuropathy (see Peripheral Neuropathy, [[Peripheral Neuropathy]])
    • 2 cases had resolution of neuropathy with treatment of bronchiectasis
  • Primary Ciliary Dyskinesia (see Primary Ciliary Dyskinesia, [[Primary Ciliary Dyskinesia]])
  • Pulmonary Sequestration (see Pulmonary Sequestration, [[Pulmonary Sequestration]])
  • Rheumatoid Arthritis (RA) (see Rheumatoid Arthritis, [[Rheumatoid Arthritis]])
  • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
  • Swyer-James-Macleod Syndrome (see Swyer-James-Macleod Syndrome, [[Swyer-James-Macleod Syndrome]])
  • Unilateral Pulmonary Artery Agenesis (see Unilateral Pulmonary Artery Agenesis, [[Unilateral Pulmonary Artery Agenesis]])
  • Williams-Campbell Syndrome (see Williams-Campbell Syndrome, [[Williams-Campbell Syndrome]]): congenital cartilage deficiency
  • Yellow Nail Sydrome (see Yellow Nail Syndrome, [[Yellow Nail Syndrome]])

Physiology

  • General Features
    • Destruction of muscular and elastic components of medium-sized bronchial walls by mediators released by neutrophils (elastase) and monocytes (cytokines) -> >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 (shown by radiolabelled aerosol studies): predisposes to bacterial airway colonization and infection
  • Specific Role of Infection
    • It is not clear whether mycobacterial infection is a cause or a consequence of bronchiectasis [MEDLINE]
    • Pseudomonas infection appears to be asscoiated with more severe disease and cystic bronchiectasis
    • Mycobacterium Avium-Intracellulare (MAI) 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

Reversibility of 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

  • Can be diffuse or localized
  • Posterior-basal segments: most commonly involved
  • Bilateral LL involvement occurs in 33% of cases/ unilat-eral LL involvement occurs in left and right lungs with equal frequency/ 50% of patients with LLL involvement also have lingular involvement
  • RML is more commonly affected than RUL
  • UL involvement most common in posterior and apical segments (and is usually due to [[Allergic Bronchopulmonary Aspergillosis]] and [[Tuberculosis]])

Pathologic Classification (Reid)

  • There is no epidemiologic or etiologic significance to this classification
    • Cylindrical: consistent widening of segments
    • Varicose: local constrictions in cylindrical segments (resembling varicsoe veins)
    • Saccular/Cystic: dilatation increases toward lung periphery (forming sacs)

Diagnosis

Arterial Blood Gas (ABG) (see Arterial Blood Gas, [[Arterial Blood Gas]])

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

Sputum Gram Stain and Culture

  • Classic 3-Layered Sputum
    • Top: frothy watery layer
    • Middle: turbid mucopurulent layer
    • Bottom: purulent opaque layer (bottom layer may also contain Dittrich’s plugs and/or yellow or white concretions)
  • Culture
    • Pseudomonas Aeruginosa (see Pseudomonas Aeruginosa, [[Pseudomonas Aeruginosa]]): colonizes bronchiectasis patients in 33% of cases
    • Haemophilus Influenzae (see Haemophilus Influenzae, [[Haemophilus Influenzae]]): common
    • Other Gram-Negatve Rods: common
    • Staphylococcus Aureus (see Staphylococcus Aureus, [[Staphylococcus Aureus]]): may be seen in some cases
    • Streptococcus Pneumoniae (see Streptococcus Pneumoniae, [[Streptococcus Pneumoniae]]): may be seen in some cases
    • Non-Tuberculous Mycobacteria: commonly cultured in North American patients
    • Mycobacterium Avium-Intracellulare (MAI) (see Mycobacterium Avium Complex, [[Mycobacterium Avium Complex]]): most common mycobacteria found in bronchiectasis patients
    • Mycobacterium Abscessus (see Mycobacterium Abscessus, [[Mycobacterium Abscessus]])

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]])

  • Most studies are flawed, since concurrent COPD cannot be excluded (however, many cases have obstruction)
  • 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, [[Bronchoscopy]])

  • Airways may appear wider on visual examination
  • Useful to evaluate for sources of hemoptysis, rule out foreign bodies, etc.
  • TBB: not usually performed

Chest X-Ray (CXR)/Chest CT Patterns

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

High-Resolution Chest CT (HRCT) (see High-Resolution Chest CT, [[High-Resolution Chest CT]])

  • Considered the gold standard imaging modality
  • Sensitivity: 84%
  • Specificty: 82%
  • Bronchial wall thickening and dilatation

Bronchography

  • No longer used

Alpha-1 Anti-Trypsin Level

  • To rule out alpha-1 antitrypsin deficiency

Immunoglobulin Levels

  • To rule out immunodeficiency

Sweat Test

  • To rule out cystic fibrosis

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

  • To rule out ciliary disorder

Clinical Manifestations

Otolaryngologic Manifestations

  • Recurrent/Chronic Rhinosinusitis: may be seen in association with bronchiectasis in particular in disorders with B-cell dysfunction

Pulmonary Manifestations

  • Acute/Chronic Hypoventilation (see Acute Hypoventilation, [[Acute Hypoventilation]] and Chronic Hypoventilation, [[Chronic Hypoventilation]]): hypoventilation typically occurs only in advanced cases or in those with superimposed COPD
  • Cough with or without Sputum Production (see Cough, [[Cough]]): most common symptom
    • “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: more common in extensive disease and in those with superimposed COPD
  • Empyema (see Pleural Effusion-Parapneumonic, [[Pleural Effusion-Parapneumonic]])
  • Hemoptysis (see Hemoptysis, [[Hemoptysis]])
    • More common in dry bronchiectasis
    • Usually mild, but may be massive (>250 mL/day)
    • Rarely fatal
  • Lung Abcess (see Lung Abscess, [[Lung Abscess]])
  • Pneumothorax (see Pneumothorax, [[Pneumothorax]])
  • Pulmonary Hypertension/Cor Pulmonale (see Pulmonary Hypertension, [[Pulmonary Hypertension]])
    • Epidemiology: 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
  • Recurrent Pneumonia (see Pneumonia, [[Pneumonia]])
  • Wheezing

Rheumatologic Manifestations

  • Clubbing (see Clubbing, [[Clubbing]])
    • Physiology: correlation between augmented vascular supply in lungs and vascularity in clubbed digits of bronchiectasis patients (suggests a possible vasodilator substance that is not yet identified)

Other Manifestations

  • Amyloidosis (see Amyloidosis, [[Amyloidosis]]): rarely seen today in developed countries
  • Brain Abscess (see Brain Abscess, [[Brain Abscess]])
  • Fever (see Fever, [[Fever]])
  • Generalized Weakness
  • Weight Loss (see Weight Loss, [[Weight Loss]])

Treatment

Treatment of Hemoptysis

  • Bronchoscopy (see Bronchoscopy, [[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, [[Spinal Cord Infarction]])
  • Surgery: may be required in some cases for refractory hemoptysis

Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin, [[Intravenous Immunoglobulin]])

  • Prevents Exacerbations in the Setting of Immunoglobulin Deficiency

Postural Drainage

  • xxx

Chest Physical Therapy (PT)

  • xxx

Humidification

  • xxx

Surgery

  • Indications: localized bronchiectasis with recurrent infection unresponsive to antibiotics

Mucolytics

  • General Comments: coughing is probably superior
  • Deoxyribonuclease (DNase) (see Dornase Alfa, [[Dornase Alfa]]): useful only for cystic fibrosis, as trials suggest that it has potential harmful effects in non-cystic bronchiectasis [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, [[N-Acetylcysteine]]): further studies are required

Antibiotics

Clinical Utility

  • Antibiotic Treatment of Acute Exacerbation: antibiotics are standard therapy
  • Antibiotic Maintenance Therapy: indicated for patients with >2-3 exacerbations per year

Agents

  • Fluoroquinolones (see Fluoroquinolones, [[Fluoroquinolones]])
    • Ciprofloxacin (Cipro) (see Ciprofloxacin, [[Ciprofloxacin]])
    • Levofloxacin (Levaquin) (see Levofloxacin, [[Levofloxacin]])
    • Ofloxacin (see Ofloxacin, [[Ofloxacin]])
    • 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, [[Gentamicin]])
    • Randomized Trial of Nebulized Gentamicin in Non-Cystic Bronchiectasis (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
  • Macrolides (see Macrolides, [[Macrolides]])
    • Azithromycin (see Azithromycin, [[Azithromycin]])
    • Erythromycin (see Erythromycin, [[Erythromycin]])
    • EMBRACE Trial (2012) [MEDLINE]: azithromycin x 6 mo -> decreased rate of exacerbations (in patients who had at least one exacerbation in the past year)
    • BAT Trial (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 (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 (2014) [MEDLINE]: long-term erythromycin treatment changes the composition of respiratory microbiota in patients with bronchiectasis
      • In patients without Pseudomonas Aeruginosa airway infection, erythromycin did not significantly reduce 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
  • Penicillins (see Penicillins, [[Penicillins]])
  • Tetracyclines (see Tetracyclines, [[Tetracyclines]])
  • Sulfamethoxazole-Trimethoprim (Bactrim, Septra) (see Sulfamethoxazole-Trimethoprim, [[Sulfamethoxazole-Trimethoprim]])

Other Treatments

  • Nutrition: indicated for weight loss
  • Inhaled Indomethacin (see Indomethacin, [[Indomethacin]]): decreases bronchorrhea (anti-inflammatory)
  • Bronchodilators: terbutaline increases tracheal mucous velocity in CF patients
  • Oxygen (see Oxygen, [[Oxygen]]): probably useful (if required)

Prevention of Bronchiectasis

  • Vaccination
  • Prompt Removal of Foreign Bodies in Airway

Prognosis

  • Prognosis was Previously Poor in the Pre-Antibiotic Era: however, the current prognosis is comparable to that of COPD
    • 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]

Treatment

  • A randomized controlled trial of nebulized gentamicin in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med 2011;183:491-499 [MEDLINE]
  • Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial. Lancet. 2012 Aug 18;380(9842):660-7 [MEDLINE]
  • Effect of long-term, low-dose erythromycin on pulmonary exacerbations among patients with non-cystic fibrosis bronchiectasis: the BLESS randomized controlled trial. JAMA. 2013 Mar 27;309(12):1260-7 [MEDLINE]
  • Effect of azithromycin maintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: the BAT randomized controlled trial. JAMA. 2013 Mar 27;309(12):1251-9 [MEDLINE]
  • 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]