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Bronchiectasis

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					Bronchiectasis
Presentation
Sir, this patient has got bronchiectasis affecting both lower lobes as evidenced by late,
coarse inspiratory crepitations heard best posteriorly in the lower one third bilaterally.
Patient has a productive cough with large volume of purulent sputum with hemoptysis
associated with clubbing.

Chest excursion was reduced bilaterally with a normal percussion note and vocal
resonance. Trachea is central and the apex beat is not displaced.
There are no signs to suggest presence of COPD.

(There is concomitant COPD with a reduced chest excursion bilaterally, hyperinflation of
the chest associated with hyperresonance on percussion with loss of liver and cardiac
dullness. There is presence of ronchi and a prolonged expiratory phase. Vocal resonance
is normal. Trachea is central and apex beat is not displaced.)

There is complication of pulmonary hypertension with a loud and palpable component of
the second heart sound associated with a left parasternal heave. There is also cor
pulmonale with a raised JVP of 3 cm with prominent a wave associated with bilateral
pedal oedema. Clinically there are no signs of polycythemia such as plethoric facies or
conjunctival suffusion.

He is not in respiratory distress (with a RR of 14 bpm without use of accessory muscles
of respiration). There are no signs of respiratory failure (he does not require any
supplemental oxygen and there is no central cyanosis; there is also no flapping tremor of
the hands and no bounding pulse). There is also no nicotine staining of the fingers,
patient is not cachexic looking and no enlarged Cx LNs.

With regards to aetiology, there is no dextrocardia or a nasal voice to suggest possible
Kartagener’s syndrome. In addition, there is no symmetrical deforming polyarthropathy
to suggest RA or any cutaneous signs of SLE. There is no kyphoscoliosis.

With regards to treatment, patient has a steroid metered-dose inhaler, salbutamol and
ipratropium metered-dose inhalers by the bed side.

I would like to complete the examination by looking at the temperature chart for fever as
well as an abdominal examination to look for splenomegaly from amyloidosis which can
result from bronchiectasis. A neurological examination is useful to screen for deficit as
patients are prone to brain abscesses.

In summary, this patient has bronchiectasis affecting both lower lobes with complications
of pulmonary hypertension and cor pulmonale. There is no concomitant COPD and no
polycythemia. He is clinically not in respiratory failure. The possible causes for this
patient’s bronchiectasis are post infective causes such as post viral, bacterial, TB or
ABPA, connective tissue disease such as RA or SLE, congenital conditions such as cystic
fibrosis, Kartagener’s syndrome or hypogammaglobulinemia.
Questions
What are your differential diagnoses for a patient that is clubbed and has crepitations?
  o Bronchiectasis
  o Pulmonary fibrosis
  o Mitotic lung lesion
  o Abscess

What is bronchiectasis?
  o Definition: permanent dilatation of the bronchi
  o Pathology: Retained secretions and chronic inflammation
  o Clinical course: Chronic, progressive with recurrent infective exacerbations
  o Clinical: Symptoms - productive purulent cough, dyspnea and hemoptysis and
       Signs: coarse late inspiratory crepitations with a 3 layered purulent sputum

What are the causes of bronchiectasis?
  o Focal
           o Luminal blockage – FB, broncholith
           o Arising from the wall – mitotic lesion of the lung
           o Extrinsic – enlarged LNs esp middle lobe from TB/fungi; displacement of
              airways post lobar resection
  o Diffuse
           o Post infectious conditions
                   Bacteria – Pseudomonas, Hemophilus, Pertussis
                   TB
                   Aspergillus (for upper lobe or proximal bronchiectasis) as in
                      allergic bronchopulmonary aspergillosis from type III immune
                      complex reactions.
                   Virus – adenovirus, measles, influenza
           o Congenital conditions
                   Cystic fibrosis
                   Alpha 1 Antitrypsin deficiency
                   Kartagener’s syndrome of immotile ciliary syndrome
                   Hypogammaglobulinemia
                          o NB: Immunodeficiency form secondary causes such as
                              cancer, chemotherapy or immune modulation post
                              transplant
           o Rheumatic conditions
                   RA (1-3% of patients)
                   SLE
                   Sjogren’s
           o Others
                   Yellow nail syndrome (yellow nails, bronchiectasis, pl effusion
                      and lymphedema)
                     Young’s syndrome(secondary ciliary dyskinesia from mercury
                      intoxication)
                     Inflammatory bowel disease (UC or Crohn)
                     Congenital kyphoscoliosis
                     Idiopathic (50%)

What is bronchiectasis sicca?
  o “dry” bronchiectasis
  o Presents with recurrent hemoptysis and dry cough
  o Affects the upper lobes therefore good drainage
  o Usually from past history of granulomatous infection eg TB

What is Kartagener’s syndrome?
  o It is a type of immotile ciliary syndrome
  o Comprising of
          o dextrocardia, situs inversus
          o bronchiectasis, sinusitis, frontal sinus dysplasia, otitis media
          o infertility
  o Resulting in poor ciliary function with retained secretions and recurrent infections
       and thus bronchiectasis

What is cystic fibrosis?
  o Most commonly due to mutations to CFTR (CF transmembrane conductance
       regulator) with F508
  o Recurrent respiratory infections with pancreatic exocrine deficiency and short
       stature
  o Upper lobe involvement
  o Staph aureus, Ps aeuroginosa
  o Elevated sweat Na and Cl concentrations

What are the differences in bronchiectasis vs COPD?
    o They may both occur concomitantly
                                COPD                           Bronchiectasis
Cause                          Cigarette                      Infection, genetic
Infection                     Secondary                            Primary
Organism               S. pneumoniae, Haem                  Haem, Pseudomonas
Symptoms              Dyspnea, chronic cough           Dyspnea, hemoptysis, productive
Sputum                      Mucoid clear                     3 layered, purulent
CXR                 Hyperlucency, hyperinflated          Airway thickening, dilated

What are the complications of bronchiectasis?
  o Pneumonia, collapse, pleural effusion, lung abscess, pneumothorax, hemoptysis
  o Brain abscess
  o Sinusitis
  o Amyloidosis
How would you investigate?
The diagnostic investigation of choice is a HRCT but simple Ix such as CXR and LFT are
also useful:
    o CXR – Diagnosis, extent and complications
            o 90% abnormal
            o Diagnosis
                   specific
                           dilated and thickened airways
                           Ring shadows (seen on end)
                           Tram lines
                   Non-specific
                           Linear or plate-like atelectasis
                           Scattered irregular opacities
                           Focal pneumonitis
            o Extent and distribution
            o Complications
                   Pneumonia, abscesses, pleural effusion
    o Lung function test
            o Obstructive pattern with FEV1/FVC <70%
            o Severity of obstruction based on FEV1
            o Reversibility with beta agonist
                   40% of patients have >15% improvement
    o High-resolution computer tomography scan of the thorax
            o Non-contrast study with 1 mm cuts every 1 cm with acquisition time of
              one second during full inspiration (requires patient cooperation); 90%
              sensitivity
            o Diagnostic
                   Dilatation of airway lumen >1.5X cf to a nearby vessel
                   Signet ring sign (dilated bronchus with its pulmonary artery)
                   Lack of tapering of an airway toward the periphery with presence
                      of bronchi within 1 cm from the pleura
                   Reid’s classifications
                           Cylindrical or tubular
                           Varicose
                           Saccular or cystic
                   Useful also in elucidation cause of focal bronchiectasis
            o Assess distribution
                   Usually lower lobes
                   If upper lobes – suspect Cystic fibrosis or ABPA
                   If proximal bronchiectis, ABPA
                   If ML or lingula – M. avium complex
            o Complications

How would you manage?
  o Non-Pharmacological
     o Education and counselling
    o Stop smoking, vaccinations (yearly influenza and 3-yearly pneumococcal)
    o Chest percussion and postural drainage (no evidence actually)
  o Rx underlying cause
  o Pharmacological
    o Rx acute exacerbations
           o O’Donnell’s 4/9 symptoms of exacerbations
                    Increased dyspnea
                    Increase cough
                    Increase sputum production
                    Increased wheezing
                    Fever
                    Lethargy, malaise
                    Changes in chest sounds
                    Reduced pulmonary function
                    Radiographic changes consistent with a new pulmonary
                       process
           o Antibiotics targeting
                    Haem, Ps and Strep and Moraxella
                    Fluoroquinolones
                    Others
                           MAC – Rifampiciin, ethambutol and Azithro till c/s
                               negative for 1 year
                           ABPA – augmentation of corticosteroids and use of
                               itraconazole 200mg bd for 4 weeks then 200mg om for
                               4 more weeks
    o Bronchodilator therapy such as beta agonists and anticholinergics with inhaled
       corticosteroids
           o Improve lung function (FEV1) and reduce sputum volume
           o No effect on mortality
    o Aerosolised recombinant human DNAse for cystic fibrosis (not for other
       causes of bronchiectasis)
o   Surgery
    o Focal
           o Removal of obstructing tumour or FB
    o Diffuse
           o Segments that are most damaged and contributing to recurrent acute
               exacerbations
           o Segments involved with uncontrolled haemorrhage
           o Removal of segments suspected of harbouring drug resistant organism
               such as MDR MTB or MAC
    o Lung transplant
How do you manage complication of hemoptysis?
  o Quantify
         o If >600mls /day = massive
  o Lie on the affected side
  o Protect airway
  o Bronchoscope or CT to determine site of bleed
  o Interventional radiology or surgical removal

				
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