What Is Bronchiectasis, Causes, Symptoms, Treatment

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What Is Bronchiectasis/Bronchiectasis is a chronic condition characterized by permanent and irreversible dilatation of the bronchial airways and impairment of mucociliary transport mechanism due to repeated infection leading to colonization of organism and pooling of the mucus in the bronchial tree. Bronchiectasis is responsible for the significant loss of lung function and one that can result in considerable morbidity and even early mortality.

Bronchiectasis describes a permanent dilation of the bronchi and bronchioles as a result of the destruction of the muscles and elastic connective tissues.

Bronchiectasis is an abnormal widening of one or more airways. Normally, tiny glands in the lining of the airways make a small amount of mucus. Mucus keeps the airways moist and traps any dust and dirt in the inhaled air. Because bronchiectasis creates an abnormal widening of the airways, extra mucus tends to form and pool in parts of the widened airways. Widened airways with extra mucus are prone to infection.

Another Name

  • Acquired bronchiectasis
  • Congenital bronchiectasis

Causes of Bronchiectasis

Causes of bronchiectasis

CauseMean incidenceSupporting diagnostic featuresDiagnostic investigations
Cystic fibrosis0.6%–2.7%Younger age (< 45 yr); the history of malabsorption; the history of pancreatitis; the history of Pseudomonas aeruginosa infection, Staphylococcus aureus infection, nontuberculous mycobacterial infection; the history of male infertilitySweat chloride assessment; CFTR genetic analysis as per guidelines (specialist center referral)
Alpha1-antitrypsin deficiency0.6%–11.3%Evidence of emphysema; obstructive pattern on spirometry; panniculitisSerum alpha1-antitrypsin level; phenotyping in those with low serum levels
Primary ciliary dyskinesia2.0%–10.3%History of chronic upper respiratory tract problems, otitis media, male infertility; abnormal ciliary beat pattern ± frequency on nasal brushingsMeasurement of nasal nitric oxide levels; ciliated epithelial biopsy (specialist center referral)
Allergic bronchopulmonary aspergillosis0.9%–7.8%History of asthma; peripheral blood eosinophils > 500 cells/μL; positive Aspergillus fumigatus IgG or positive precipitins; sputum culture of A. fumigatus; fleeting infiltrates on chest radiograph or CT chest; proximal bronchiectasis on CT chest scanTotal IgE > 500 IU/mL; positive A. fumigatus–specific IgE or immediate reaction on skin-prick testing
Autoimmune/connective tissue diseases (typically rheumatoid arthritis, SLE)1.8%–31.1%History or clinical signs of connective tissue disease ± vasculitisRheumatoid factor; anti-CCP; other investigations pertinent to suspected diagnosis from a clinical review
Inflammatory bowel diseases1.0%–3.0%History or clinical signs of ulcerative colitis or Crohn diseaseSpecialist gastrointestinal review; positive pathological features on colonoscopy
Congenital malformations0.2%–0.6%Williams–Campbell syndrome (bronchomalacia); Mounier-Kuhn syndrome (tracheobronchomegaly) and lung sequestrationTypically diagnosed on chest CT
Aspiration0.2%–11.3%History of reflux; the history of aspirationVarious modalities available: videofluoroscopic swallow study; upper gastrointestinal endoscopy; ambulatory esophageal manometry; pH studies; flexible endoscopic evaluation of swallow
Humoral immunodeficiency1.1%–16.0%History of recurrent infectionsSerum immunoglobulins levels (IgG, IgA, and IgM); specific antibody responses to pneumococcal, Haemophilus influenzae B and tetanus antigens
Postinfectious29.0%–42.0%History or radiologic evidence of the previous infection (e.g., frequently, pneumonia, Bordetella pertussisMycobacterium tuberculosis, nontuberculous mycobacteria)
Idiopathic26.0%–53.0%Other causes excludedOther causes excluded

Historically, the most common cause of bronchiectasis was thought to be an antecedent respiratory infection, often during childhood. The causes are idiopathic, acquired, or infection-related.

  • Post infective (post pneumonia, whooping cough, measles, mycobacterial infection)
  • Mucociliary disorder (immotile cilia, Kartagener’s syndrome, Young’s syndrome)
  • Obstructive (foreign body, mycobacterial infection, obstructing cancer)
  • Immune disorder (hypogammaglobulinemia, HIV infection, cancer, allergic bronchopulmonary aspergillosis, transplant rejection)
  • Rheumatic/inflammatory disease (rheumatoid arthritis, inflammatory bowel disease)
  • Extremes of age
  • Malnutrition/socioeconomic disadvantage
  • Chronic obstructive pulmonary disease\
  • Miscellaneous (yellow nail syndrome)
  • Alpha1-antitrypsin deficiency

Bacterial Infections

  • Mycobacterium: Tuberculosis and atypical
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Staphylococcus aureus
  • Mycoplasma and HIV

Viral Infections

  • Respiratory syncytial virus and measles

Fungal Infections with Bronchial obstruction

  • Foreign body
  • Mucus plug
  • Tumors
  • Hilar lymphadenopathy (right middle lobe syndrome: extrinsic compression from postinfectious adenopathy)

Postinflammatory pneumonitis

  • Chronic aspiration/gastroesophageal reflux disorder
  • Chronic sinusitis
  • Inhalational injury

Congenital/Genetic

  • Cystic fibrosis
  • Young syndrome
  • PCD: primary ciliary dyskinesia (Kartenger Syndrome)
  • immunodeficiency (hypogammaglobulinemia)
  • Alpha1-antitrypsin deficiency (AAT)
  • Mounier-Kuhn syndrome

Inflammatory diseases

  • Ulcerative colitis
  • Rheumatoid arthritis
  • Sjögren syndrome

Pulmonary Diseases

  • Asthma
  • Bronchomalacia
  • Cronic-obstructive pulmonary disease (COPD) (reported in up to 50% of patients with moderate-to-severe COPD)
  • Diffuse panbronchiolitis
  • Idiopathic pulmonary fibrosis (traction bronchiectasis)

Altered immune response

  • Allergic Bronchopulmonary Aspergillosis
  • Hypersensitivity pneumonitis

Others

  • Yellow nail syndrome (yellow nails, lymphedema, pleural effusion, bronchiectasis 40% first described in 1964)
  • Allergic bronchopulmonary aspergillosis (ABPA) is a rare but typical complication in bronchiectasis. The underlying pathophysiology is sensitization to Aspergillus fumigatus, which leads to a CD4+/TH2 mediated inflammatory reaction.
  • Typical symptoms include a raised body temperature, weight loss, a drop in FEV1, and pulmonary infiltrates on the radiograph. Bronchiectasis can be a sequela of ABPA, but it can also predispose to ABPA.

Etiology of bronchiectasis modified from []
CategoryCause
Postinfectious
  • Viral

  • Bacterial

  • Fungal

  • Atypical mycobacteria

ABPA
COPD
Idiopathic traction
  • Posttuberculous fibrosis

  • Radiation fibrosis

  • Pulmonary fibrosis

Aspiration
  • Foreign body aspiration

Obstruction
  • Benign tumors

  • Enlarged lymph nodes

Amyloidosis
Celiac disease
Yellow nail syndrome
Young’s syndrome
Immunological defectsPrimary

  • CVID

  • Agammaglobulinemia

  • Hyper-IgE syndrome

Secondary

  • Chemotherapy

  • Immunosuppressant therapy

  • Tumor

Congenital defectsAnatomical

  • Scoliosis

  • Marfan syndrome

  • Tracheobronchomegaly

Others

  • Primary ciliary dyskinesia

  • Kartagener syndrome

  • Alpha-1-antitrypsin deficiency

  • defect ENaC protein

Diffuse panbronchiolitis
Rheumatoid arthritis
Systemic lupus erythematosus
Chronic bowel disease

ABPA, allergic bronchopulmonary aspergillosis; COPD, chronic obstructive pulmonary disease;

ENaC, epithelial sodium channel; CIVD, common variable immunodeficiency

Acquired causes

  • Bronchiectasis secondary to a large carcinoid tumor (not shown) that was completely obstructing the bronchus proximally. The yellowish discoloration of lung parenchyma reflects obstructive pneumonia.
  • Tuberculosis, pneumonia, inhaled foreign bodies, allergic bronchopulmonary aspergillosis and bronchial tumors are the major acquired causes of bronchiectasis.[rx][rx] Infective causes associated with bronchiectasis include infections caused by the StaphylococcusKlebsiella, or Bordetella pertussis, the causative agent of whooping cough and nontuberculous mycobacteria.[rx][rx]
  • Aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), various allergies all appear to be linked to the development of bronchiectasis.[rx]
  • Childhood Acquired immune deficiency syndrome (AIDS), which predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infections.[rx]
  • Inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn’s disease as well but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungal spores.[rx] A Hiatal hernia can cause Bronchiectasis when the stomach acid that is aspirated into the lungs causes tissue damage.
  • People with rheumatoid arthritis who smoke appear to have a tenfold increased rate of the disease.[rx] Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis.
  • Case reports of Hashimoto’s thyroiditis and bronchiectasis occurring in the same persons have been published.[rx]

Congenital causes

  • Bronchiectasis may result from congenital disorders that affect cilia motility or ion transport.[rx] Kartagener syndrome is one such disorder of cilia motility linked to the development of bronchiectasis.[rx]
  • A common cause is a cystic fibrosis, which affects chloride ion transport, in which a small number of patients develop severe bronchiectasis.[rx]
  • Young’s syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic infections of the sinuses and bronchiole tree.[rx]

Symptoms of Bronchiectasis

  • Cough – 98%, sputum: 78% (sputum is typically mucoid and relatively odorless), dyspnea: 62%, haemoptysis: 56% to 92%, and pleuritic chest pain: 20% (secondary to chronic coughing).
  • Some people with bronchiectasis – may have a cough productive of frequent green/yellow mucus (sputum), up to 240 ml (8 oz) daily.
  • Bronchiectasis – may also present with coughing up blood (hemoptysis) in the absence of sputum, called “dry bronchiectasis”. Sputum production may also occur without coloration. People with bronchiectasis may have bad breath indicative of active infection.
  • Frequent bronchial infections – and breathlessness are two possible indicators of bronchiectasis. Crepitations and expiratory rhonchi may be heard on auscultation. Nail clubbing is rare.[rx]
  • Chronic daily cough
  • Coughing up blood
  • Abnormal sounds or wheezing in the chest with breathing
  • Shortness of breath
  • Chest pain
  • Coughing up large amounts of thick mucus every day
  • Weight loss
  • Fatigue
  • Change in the structure of fingernails and toenails, known as clubbing
  • Frequent respiratory infections
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Diagnosis of Bronchiectasis

  • Blood tests – to check how well your immune system is working and check for infectious agents, such as bacteria, viruses and fungi
  • Phlegm (sputum) test – to check for bacteria or fungi
  • a sample of your sweat can be tested to see how much salt is in it – high levels of salt can be caused by cystic fibrosis (if this test is positive, a more detailed genetic test can be carried out.
  • Lung function test – a small, handheld device (a spirometer) that you blow into is used to measure how hard and how quickly you can expel air from your lungs; this can assess how well your lungs are working
  • Bronchoscopy – a flexible tube with a camera at one end is used to look into your lungs; this is usually only required if you think you have inhaled a foreign object

Often patients are diagnosed after many years of symptoms when a chronic cough or hemoptysis become debilitating.

General findings may include digital clubbing (2% to 3%), cyanosis, plethora, wasting, and weight loss.

  • Local chest examination –  Most commonly crackles and wheezes on auscultation.
  • Crackles – 75%, usually bi-basal.
  • Wheezing – 22% (wheezing may be due to airflow obstruction from secretions)

Clinical Features of Associated Causative condition: 

  • Connective tissue diseases – Arthritis Sicca syndrome
  • ABPA – Prominent wheezing
  • Bronchial obstruction – localized wheezing
  • PCD, CF, Young Syndrome – Recurrent sinus disease, infertility
  • Features of acute exacerbation of bronchiectasis – Change in sputum production, increased dyspnea, increased cough, fever, increased wheezing, and reduced pulmonary function.

The British Thoracic Society (BTS) guidelines recommend testing for underlying causes including measurement of immunoglobulins (IgA, IgM, IgG, and IgE), testing to exclude ABPA (specific IgE to Aspergillus, IgG to Aspergillus and eosinophil count).

  • Sputum culture to exclude nontuberculous mycobacteria (NTM) – and measurement of autoantibodies are also suggested. Testing for cystic fibrosis (CF) (sweat test and/or screening for common CF mutations) is recommended for patients aged younger than 40 years or with recurrent Pseudomonas aeruginosa and Staphylococcus aureus isolation, or upper lobe predominant disease irrespective of age.
  • Alpha-1 Antitrypsin level –  Ciliary function analysis, and Serology for HIV  if indicated.

Imaging

Chest radiography is usually the initial study performed in suspected bronchiectasis.

  • Signs on chest x-ray include the identification of parallel linear densities, tram-track opacities, or ring shadows reflecting thickened and abnormally dilated bronchial walls.
  • Signs of complications/exacerbations, such as patchy densities due to mucoid impaction (mucus may become of high density due to chronic inspissation) and consolidation, volume loss secondary to mucoid bronchial obstruction or chronic cicatrization are also seen.
  • In comparison to chest x-ray, CT is both more sensitive and provides more specific information. In addition to making the diagnosis, the pattern of disease on HRCT may enable one to limit the differential to a single/few specific causative entities.

The CT chest signs of bronchiectasis were first described by NAIDICH

Bronchial dilation, the cardinal sign of bronchiectasis, is characterized

  • On HRCT by a broncho arterial ratio (BAR) of more than 1
  • Lack of bronchial tapering (normal airways diminish in caliber as they extend toward the lung periphery)
  • Visibility of airways within 1 cm of the pleural surface (normal airways should not be visualized this far out in the lung periphery) or abutting the mediastinal pleural surface.

Usual Pattern on High-Resolution CT scan Chest

  • Cylindrical (tram track sign)  Dilated airways seen in a horizontal orientation.
  • Signet-ring – The dilated airway lies adjacent to a pulmonary artery branch giving the appearance of a ring (internal bronchial diameter greater than that of the adjacent pulmonary artery).
  • Varicose  Implies nonuniform bronchial dilatation. The bronchi resemble like varicose veins. The luminal dilatation is characterized by alternating areas of luminal dilatation and constriction, creating a beaded appearance, and the wall thickening is irregular. This varicose bronchiectasis serves as an intermediate step before the development of grossly dilated, cystic airways.
  • Cystic or saccular  A cluster of thin-walled cystic spaces.
  • Mosaic lung attenuationThis term is used to describe heterogeneous lung density due to air trapping in the affected lung segments and as a result has a geographic distribution. This finding can be produced or exaggerated on expiration.
  • Dilated bronchial arteries These are best-demonstrated post administration of intravenous contrast. These tortuous vessels extend along the central airways toward the hila. It is these vessels that are often responsible for hemoptysis.
  • Serum quantitative immunoglobulin levels – IgG, IgA, IgM
  • Sweat chloride (2 measurements) – and CF transmembrane conductance regulator (CFTR) genetic mutation analysis to exclude cystic CF – details of CF diagnosis and management are discussed elsewhere (Cystic Fibrosis)

In selected cases, one should also consider

  • IgG subclasses if immunodeficiency is suspected, despite normal total IgG
  • Alpha-1-antitrypsin level, especially in setting of concurrent basilar or early-onset emphysema in a non-smoker
  • Serum IgE level and Aspergillus specific IgE and IgG or Aspergillus precipitins
  • Autoimmune serologies, including CRP, RF, anti-CCP, ANA, SSA/B, ANCA
  • HIV test
  • Exhaled nasal nitric oxide level to exclude primary ciliary dyskinesia (PCD)

Lobar collapse

Mycetoma formation (Fungus ball) – Aspergillus fumigatus is a fungus that may colonize dilated airways or bullae/cavities. It is an important cause of hemoptysis.

Lung function

Airflow obstruction – FEV1 decreased

Air trapping – Residual volume increased

  • phlegm (sputum) test – to check for bacteria or fungi
  • a sample of your sweat can be tested to see how much salt is in it – high levels of salt can be caused by cystic fibrosis (if this test is positive, then a more detailed genetic test can be carried out

Treatment of Bronchiectasis

Chest physiotherapy

  • Effective clearance of mucus from the airways may break the vicious cycle of the disease process by decreasing the stagnation of mucus and mucus plug formation with associated bacterial colonization, recurrent infection, and inflammation.
  • Chest physiotherapy has been used for many years and a number of techniques are available for mobilizing secretions, such as postural drainage, active cycle of breathing techniques, positive expiratory pressure, oscillatory positive expiratory pressure devices, and high-frequency chest wall percussion. One recent, small, randomized control trial concluded that regular chest physiotherapy using an oscillatory positive expiratory pressure device in patients with non-cystic fibrosis bronchiectasis had small, but significant benefits in improving cough severity, exercise capacity, and SGRQ total score despite earlier negative systematic reviews,.
  • Physiotherapy and airway clearance exercises can help to clear mucus (visit the Bronchiectasis Patient Priorities website for advice and video demonstrations)
  • Medications can help to clear mucus (mucoactive drugs). These should be combined with regular physiotherapy exercises to be most effective.
  • Bronchodilator inhalers relax the muscles in the airway.
  • Regular exercise helps to clear sputum and encourages the lungs to work better.

Improve tracheobronchial clearance – Most physicians recommend mucus clearance as the mainstay of therapy in bronchiectasis, Postural drainage consists of adopting a position in which the lobe to be drained is uppermost, and should be performed for a minimum of 5 to 10 minutes twice a day. Efficiently performed, this is of great value both in reducing the amount of cough and sputum and in preventing recurrent episodes of bronchopulmonary infection.

  • Deep breathing followed by forced expiratory maneuvers (the active cycle of breathing technique) is of help in allowing secretions in the dilated bronchi to gravitate towards the trachea, from which vigorous coughing can clear them. Percussion of the chest wall with cupped hands may help to dislodge sputum, and a number of mechanical devices are available which cause the chest wall to oscillate, thus achieving the same effect.

Bronchiectasis is treatable but rarely curable.

Treatment Goals:

  • Identifying and treating the underlying cause
  • Improve tracheobronchial clearance
  • Control infection
  • Reverse airflow obstruction

Identifying and treating the underlying cause – Immunoglobulin replacement, steroids, and antifungals for ABPA, treatment for NTM, and of CF all represent opportunities to treat the underlying cause specifically, and systematic testing of all patients is recommended in consensus guidelines.

  • Control infection – Choice of antibiotic should primarily be based on the results of culture and sensitivity. When no specific pathogen is identified, and the patient is not seriously ill, an oral agent like amoxicillin, co-amoxiclav, or macrolides for 2 weeks is sufficient.
  • Use a higher dose of oral amoxicillin 1 gm twice per day for 2 weeks, especially if colonized with H. influenza, if pseudomonas-colonized then a 2-week course of ciprofloxacin 750 mg twice per day (with cautious use in the elderly) is reasonable.
  • For patients with moderate-to-severe symptoms, parenteral antibiotics, such as an aminoglycoside (gentamicin, tobramycin) and antipseudomonal synthetic penicillin, a third-generation cephalosporin, or a fluoroquinolone, may be indicated.
  • Treatment for Pseudomonas isolates 2 weeks of intravenous (IV) antipseudomonal antibiotics, nebulized colistin for 3 months, or nebulized colistin for 3 months with an additional 4 weeks of oral ciprofloxacin.
  • Maintenance therapy with intermittent antibiotics is not used routinely in patients with non-CF bronchiectasis, and the decision to use long-term antibiotics should be individualized.
  • If possible, the underlying disease should be treated first. This primarily applies to immunodeficiency syndromes. In hypogammaglobulinemia, substitution treatment with immunoglobulins can be given (0.4 g/kg body weight every 4–6 weeks).
  • Inhaled aminoglycosides can be of benefit in chronic non-CF bronchiectasis; however, the treatment needs to be of sustained duration.
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Reverse airflow obstruction

  • In patients with airflow obstruction, inhaled bronchodilators and corticosteroids should be used to enhance airway patency.

General management – As with other respiratory diseases, patients with bronchiectasis should be encouraged to stop smoking. Vaccination against influenza and pneumococcal disease is also recommended.

Adjunctive Surgical Treatment

  • Surgery is only indicated in a small proportion of cases. These are usually young patients in whom the bronchiectasis is unilateral and confined to a single lobe or segment as demonstrated by CT.
  • Surgery is an important adjunct to therapy in some patients with advanced or complicated disease.
  • Single- or double-lung transplantation has been used as a treatment of severe bronchiectasis, predominantly when related to CF. In general, consider patients with CF and bronchiectasis for lung transplantation when FEV falls below 30% of the predicted value. Female patients and younger patients may need to be considered sooner.
  • Massive hemoptysis: Bronchial artery embolization and/or surgery is first-line therapy for the management of massive hemoptysis.

Airway clearance techniques and pulmonary rehabilitation

  • Chest physiotherapy forms a core of patient-led management in bronchiectasis irrespective of severity and symptoms. These techniques are patient-centered and variable and aim to aid the removal of secretions from the lung through non-pharmacological methods . Whether these techniques have a clear impact on the quality of life or reducing exacerbations has not been adequately proven, and most studies are limited to the CF patient population .

Anti-inflammatory agents

  • This broad heading covers many drugs, including corticosteroids, non-steroidal anti-inflammatory drugs (NSAIDs), leukotriene receptor antagonists, and others. All have different mechanisms of action and vary as either long- or short-term therapy.
  • NSAIDs  There is some evidence to suggest the use of ibuprofen or other NSAIDs in patients with mild CF. There are no studies that support their routine use in non-CF bronchiectasis.
  • Leukotriene receptor antagonists – There are no studies to date for this subset of therapy specific to bronchiectasis.
  • Inhaled corticosteroids +/- long-acting beta 2 agonists – The Cochrane review of the long-acting beta 2agonists (LABA) and inhaled corticosteroids (ICS) combination demonstrated a lack of quality evidence. Only one RCT (2012) was highlighted in the 2014 review evaluating a bronchiectasis adult population without asthma who received inhaled budesonide and formoterol (640µg and 18µg) or high-dose budesonide (1,600µg). The authors found that there was an improvement in dyspnoea symptoms between the combination group and the ICS group . The study size was small and it lacked statistically significant differences in outcomes .

Bronchodilator therapy

  • Beta 2 adrenoreceptor agonists (short- and long-acting beta-agonists) –  These agents have been illustrated in clinical trials for asthma and COPD; however, there have been none to date in bronchiectasis. Furthermore, there are no RCTs evaluating the use of inhaled anticholinergics. The role of bronchodilator therapy in bronchiectasis is unproven but often used in clinical practice with the breathless patient. If there is a subjective improvement, it is sensible to continue such treatment.

Bronchodilators

  • Although a large proportion of subjects with bronchiectasis have airflow obstruction with airway hyperreactivity and a significant bronchodilator response, there are no randomized, controlled trials investigating the effects of long-acting beta-agonists or anticholinergics in the management of patients with bronchiectasis,.

Mucolytics

  • Mucus retention in the lungs is a prominent feature of bronchiectasis -The stagnant mucus becomes chronically colonized with bacteria, which elicit a host neutrophilic response. This fails to eliminate the bacteria, and the large concentration of host-derived protease may contribute to the airway damage.
  • Mucolytic drugs have anti-inflammatory – antioxidant and glucoregulatory properties. Some benefits in exacerbation rate and lung function have been demonstrated in chronic obstructive pulmonary disease. Among the mucolytics, erdosteine is a thiol derivative agent approved for use in chronic obstructive pulmonary disease in some European countries. A small randomized study suggests that erdosteine decreases cough, dyspnea, and sputum in elderly patients with bronchiectasis and chronic mucus hypersecretion.

Anti-obstructive therapy

  • If a patient’s airways are obstructed, anti-obstructive treatment similar to COPD should be considered. Parasympatholytics and beta-sympathomimetics constitute the treatment of choice. Long-acting substances (tiotropium bromide or salmeterol/formoterol) seem superior to short-acting substances.
  • Proof of superiority is lacking for inhalation therapy with compression or ultrasound nebulizers (both of which are popular in Germany) compared with conventional treatment with metered-dose aerosol inhalers or powder inhalers.

Inhibiting inflammation

  • Oral corticosteroids are often administered in acute exacerbations of bronchiectasis, in analogy to acute exacerbations of COPD. For inhaled steroids, long-term usage seems to confer benefits. Tsang et al. showed in a study of 73 patients with non-CF bronchiectasis a reduction in the exacerbation rate and sputum production when using inhaled steroids [. Randomized studies are, however, lacking.
  • Macrolide antibiotics such as azithromycin have a potent anti-inflammatory effect in addition to their antibacterial effects. They reduce the production of proinflammatory cytokines. The cytokines act as chemokines for neutrophils and effect an expression of adhesion molecules, which neutrophils require for their migration from the bloodstream to the interstitium. Furthermore, in addition to their regular bacteriostatic effects, macrolides inhibit the production of biofilms by Pseudomonas aeruginosa independently of their antibiotic effectiveness[.
  • In the therapy of neutrophil-dominated, chronic inflammatory pulmonary disorders, such as diffuse panbronchiolitis (DPB) or CF, macrolides are already in use, successfully and without notable side effects.
  • The treatment with macrolide antibiotics has led to a reduction in the amount of sputum and improved 5-year survival in patients with non-CF bronchiectasis too [. At this point in time, however, neither inhaled antibiotics nor macrolides are licensed for the treatment of patients with non-CF bronchiectasis.

Allergic bronchopulmonary aspergillosis

  • Allergic bronchopulmonary aspergillosis (ABPA) is a rare but typical complication in bronchiectasis. The underlying pathophysiology is sensitization to Aspergillus fumigatus, which leads to a CD4+/TH2 mediated inflammatory reaction.
  • Typical symptoms include a raised body temperature, weight loss, a drop in FEV1, and pulmonary infiltrates on the radiograph. Bronchiectasis can be a sequela of ABPA, but it can also predispose to ABPA. The clinical diagnosis is difficult and is based on criteria set out by Greenberger (overview in Agarwal []

Surgical therapy

  • Surgery is the method of choice in unilateral and localized bronchiectasis. Several studies have shown that resection of the bronchiectasis improved symptoms [. In the different studies, mortality varied from 1% to 8.6% [, , the rate of surgical complications was 8.8–25%[.
  • Complications included pneumonia, postoperative hemorrhage, atelectasis, bronchopulmonary fistula, and wound infection. In particular cases, the resection of bilateral bronchiectasis may be the aim, but the lesions should be limited and completely resectable [. In severe complications, such as life-threatening hemorrhage or fungal infection, surgical therapy can be used as the method of last resort.
  • Hemoptysis, which is mostly caused by bleeds from hypertrophied vessels of the inflamed mucosa, can be controlled by bronchial artery embolization (coiling) if required. This should only be done in specialized centers.

Lung transplantation in advanced disease

Lung transplantation can be a useful intervention in very advanced non-CF bronchiectasis. It is of vital importance to identify the right time for putting the patient on the transplant list. In accordance with the guidelines, the following criteria should be met:

  • FEV1 < 30 % and exacerbation with inpatient admission to intensive care, or
  • More than three exacerbations per year, or
  • Recurrent pneumothorax, or
  • Hemoptysis requiring—and receiving—intervention [.
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A double lung transplant is the method of choice in more than 90% of cases. In case only one lung is transplanted, there is a risk that pathogens are transferred from the native lung into the transplanted lung.

Embolization

  • If you cough up blood, you may have a scan called bronchial angiography to look at the blood vessels in your lungs. If this shows blood vessels that cause bleeding, a procedure called embolization can block off the blood vessels causing the trouble.

Lung transplantation

  • Lung transplantation is rarely needed in bronchiectasis. It’s for people who have very severe lung disease and no other treatment options.

Oxygen

  • Your oxygen saturation levels will be checked as part of breathing tests. But most people with bronchiectasis do not require oxygen.

Vitamin D

  • There is some evidence that people with bronchiectasis can have insufficient levels of vitamin D, which is needed for healthy bones, teeth, and muscles. Your health care professional might test your vitamin D levels, especially as current government advice is that adults should have 10 micrograms of vitamin D every day.

Physiotherapy and Other Management

  • The key to management of bronchiectasis – is through education and systemic management. Currently, antibiotics are used at the first sign of change in sputum color [rx] and longterm use of antibiotics is recommended for people who experience 3 or more exacerbations a year[rx].
  • Since antibiotics – do not help with the persistent inflammation in the airways, inhaled steroids are taken as an anti-inflammatory and to decrease sputum production[rx]. Bronchodilators tend to help patients who also have co-existing COPD and asthma[rx].
  • A review and a controlled study found that many people with bronchiectasis – also have a Vitamin D deficiency and prescribing Vitamin D may have an anti-inflammatory and anti-infective role[rx][rx]. Physiotherapy has a very valuable role in aiding with symptoms of bronchiectasis. Since mucociliary clearance is reduced to about 15% of normal, patients tend to cough more[rx]. Physiotherapy treatments are aimed at aiding secretion clearance, managing fatigue induced by the effort of ineffective clearance and increased coughing. The most common and effective treatments are
  • Active Cycle of Breathing Technique (ACBT)-  is a commonly taught technique and is often used with Postural drainage and manual drainage[rx]. Its purpose is to loosen and clear excess pulmonary secretions, improve the effectiveness of cough and to improve lung ventilation and function. It consists of 3 main stages:
    • Breathing Control
    • Deep Breathing Exercises or Thoracic Expansion Exercises
    • Huffing or Forced Expiratory Technique (FET)[rx]
  • Forced expiration technique – sometimes referred to as a huff. It is part of the ACBT but can be used alone. A huff is very effective at clearing secretions, especially when combined with other airway clearance techniques.[rx]
  • Manual Therapy – is a popular treatment technique and is often used when the patient is fatigued or experiencing an exacerbation of symptoms. It describes techniques that involve external forces to the chest wall to loosen mucus and includes any combination of percussion, shaking, rib springing, vibrations and over pressure[rx]. Because of the nature of the technique, it is contraindicated in patients that are taking anticoagulants or that have osteoporosis[rx]. The aim of treatment is to:
    • Loosen secretions
    • Reduce fatigue
    • Increase the effectiveness of other treatment techniques
  • Postural Drainage (or modified postural drainage) – is an effective treatment that incorporates gravity-assisted techniques to help clear secretions from specific segments of the lungs and often requires tilting the head down to clear secretions from the middle and lower lobes.
  • PD is commonly used in conjunction with other techniques like ACBT percussion – coughing or huffing and studies have found using PD with these techniques produced a greater amount of sputum[rx][rx]. Although PD is effective in secretion clearance it is time-consuming and often found to be less tolerated than techniques that are performed in a sitting[rx].
  • Where tilting the head down is contraindicated – or not tolerated then modified postural drainage (MPD) positions can be used instead and in cases where gastroesophageal reflux disease GORD) is present and exacerbated even when using modified PD then it is suggested to use a technique that can be performed in sitting.

Autogenic Drainage is a technique that utilizes breathing control to clear secretions from the airways. The aim is to vary the depth, rate, and location of lung volumes during respiration to move secretions from the smaller airways to the larger airways for easier expectoration. It consists of three phases:

Mobilizing (unstick) phase – involves breathing as much air out of the lungs as possible and resisting the urge to cough. During this phase, crackles may be heard.

  • Collecting phase – as the secretions get louder the rate and depth of the breaths change, the speed of breathing out is faster (but not too fast to stimulate a cough) and are felt more in the middle of the chest. This assists the movement of secretions from the smaller airways to the larger airways.
  • Clearing phase – as the secretions get louder the aim is now to take full, slow deep breaths in, followed by a fast breath out. Suppress the urge to cough and after three deep breaths huff to expel the secretions. Often the huff is enough to clear secretions but if they are ineffective this is the stage where a cough may be effective in clearing secretions.
  • Positive Expiratory Pressure (PEP) – is a technique that describes breathing against resistance, and can be performed either through a device or against pursed lips. It is a technique which is recommended when other techniques have not been effective or are contraindicated; it has been found to have no adverse effect of GORD.[rx] The increase in pressure creates back pressure in the airways during expiration. This may cause a build up of gas behind the mucus, temporarily increasing functional capacity, and improving mucus clearance.[rx]
  • High-Frequency Chest Wall Oscillation – is achieved by wearing a vest that emulates chest physiotherapy. The vest applies positive pressure air pulses to the chest which causes vibrations that loosen and thin mucus, This along with an intermittent cough or huff assists with the clearance of secretions. This device allows people to perform therapy in their own time, allowing them some control and less dependence on other people.
  • Intrapulmonary Percussive Ventilation – is another technique that relies on a device to assist with the clearance of secretions. An intrapulmonary percussive ventilator (IPV) is a machine that delivers short bursts of air through a mouthpiece that vibrates the airway walls[rx]. It is indicated for short-term use when other techniques are contraindicated or have proved ineffective.
  • Intermittent Positive Pressure Breathing(IPPB) – it is an expensive piece of equipment[rx] and usually only used when all other clearance techniques have proved to be ineffective[rx]. It is commonly seen in Intensive Care and gives positive pressure as the patient breathes in, and during expiration, it creates negative pressure making the cough stronger and more effective

Physical Exercise is recommended for respiratory conditions, including bronchiectasis with the aim of improving aerobic capacity and, fitness and endurance. A study by Lee et al concluded that exercise resulted in short term improvements and had an impact on dyspnoea and fatigue, and resulted in fewer exacerbations over a 12 month period[rx].

References

What Is Bronchiectasis

 

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