1. Bronchiectasis
Khaled Al-Asad, M.D
JUH 2018 / 2019

2. Definition Bronchiectasis
is a permanent dilatation and thickening of the airways characterized by chronic cough, excessive sputum production, bacterial colonization, and recurrent acute infections
a guide for primary care. Aust Fam Physician Nov;41(11):

3. Definition
COPD
common, preventable and treatable disease that is characterized by persistent respiratory symptoms and airflow limitation that is due to airway and/or alveolar abnormalities, usually caused by significant exposure to noxious particles or gases
Asthma
Heterogeneous disease characterized by chronic airway inflammation. it is defined by history of respiratory symptoms, wheezes, cough, and shortness of breath and chest tightness that vary over time in intensity together with variable expiratory airflow limitation

4. Obstructive lung Physiology
COMMON
COPD
Asthma
Bronchiectasis
Cystic fibrosis
Uncommon / combined
Sarcoidosis
LAM
Early Scoliosis
Bronchiolitis Obliterans
Alpha Antitrypsin

5. Pathology
Dilatation : reflection of inflammatory mediators with oxidative stress, and damage of peribronchial tissue
Bronchial hypersecretion from mucus gland hypersecretions of mucus glands
Bacterial colonization ciliary dysfunction and impaired local immune response
It is
Permanent
Chronic
Persistent symptoms

6. Expected physiology Obstructive High airway resistance Air trapping
Mucus plugs
Airway disease
High airway resistance
Obstruction
Collapsibility airways
Surface area involved
Air trapping
Restrictive
Peribronchial fibrosis
Recurrent infections of parenchyma
Muscles and diaphragm dysfunction due to malnourishment

7. Pathogenesis affected airways are inflamed and easily collapse.
There is an impairment of airflow and drainage of secretions, leading to the accumulation of a large amount of mucus in the lungs.
mucus collects bacteria, predisposing to frequent and often severe LRT infections.
severity of bronchiectasis used to be classified according to the volume of sputum produced but this has now largely been superseded by using the radiological appearance on CT scan

8. Aetiology & Mechanism
The disease is caused by chronic inflammation of the airways.
Therefore
be caused by a large number of disorders which cause inflammation and infection
particularly conditions that facilitate infections
tend to be recurrent and more severe with time and cause damage to the lungs

9. Epidemiology study across 640 general practices in the UK
Prevalence in 2011 in men was 227/100,000, and 309/100,000 in women.
Incidence increased each year between 2004 and 2011, from 18/100,000 person-years at risk, to 32/100,000 person-years at risk.
Prevalence was higher in women than in men.
Prevalence was higher in the older age groups (>60 years).
Time trends in incidence and prevalence of bronchiectasis in the UK. Thorax. 2012;67

10. Discharge diagnosis USA 1993 - 2005

11. Classification
Bronchiectasis can be classified into the following forms morphologically (all three forms may be present in the same patient):
Cylindrical bronchiectasis:
bronchi are enlarged and cylindrical.
Varicose bronchiectasis:
bronchi are irregular with areas of dilatation and constriction.
Saccular or cystic:
dilated bronchi form clusters of cysts. This is the most severe form of bronchiectasis and is often found in patients with cystic fibrosis

12. Clinical presentation
Episodes of expectoration
Daily postural drainage
Putrid sputum
Cough
Dyspnea
Wheezes
Chest pain hemoptysis
Localized vs. diffuse
Crackles
Wheezes
Repeated pneumonia
Sinusitis
Situs inversus
Clubbing ?
Systemic disease

13. Sputum

14. Stages of Clubbing No visible Mild clubbing Moderate Gross HOA
Fluctuation & softening of nail beds
Loss of normal angle < 165
Lovibond angle
Schamroth sign
Apparent gross loss of the angle
Drumstick with thickening of distal part
Shiny and striation of the nail

15. Causes of clubbing Lung diseases Heart diseases GI diseases
lung CA, interstitial dis, TB, mesothelioma, lung abscess, bronchiectasis
Heart diseases
chronic hypoxia, cyanotic heart disease, SBE, A. myxoma, Tetralogy
GI diseases
malabsorption, crohn’s & UC, rimary biliary c. hepatopulmonary syndrome
Thyroid ; Grave’s
Platelet Derived Growth factor
Upregulation of PGE2

16. CAUSE Post infectious Immune defeciency Collagen vascular dis.
Gastric aspiration
ABPA, IBD
Congenital
Bronchial obstruction
Measles, TB, pertusis
HIV
RA, SLE 3-8% 4%
1-7%
Cystic F 4%, Kartagener yellow nail syndrome

17. FACT
42% of cases develop post-infection. However, there is no identifiable underlying cause in about 50% of adults and 25% of children
Characterisation of the onset and presenting clinical features of adult bronchiectasis. Respir Med Dec;100(12):2183-9

18. FACT – COPD 57.6%
elevated prevalence of bronchiectasis in patients with moderate to severe COPD
associated with severe airflow obstruction
at least one hospital admission for exacerbations in the previous year
Miguel Ángel Martínez-García – Nov 2011

19. Bronchiectasis & COPD

20. Review Natural history 842 patients Follow up 12 years
Mean admission per year 2.2
Mortality 28%
Asthma 20 %
COPD 38 %
Registry Finland
Kiestinen et al
Mortality study
Advanced age
CT involevement
Functional status
Respiratory Failure both types
Onen et al

21. FACT - pediatrics
A child with chronic respiratory symptoms and a single positive sputum culture for Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, non-tuberculous mycobacteria or Burkholderia cepacia complex
==== BRONCHIECTASIS

22. FACT - adults
Assessment of symptoms in patients with bronchiectasis should include a record of both sputum purulence and estimated or measured 24-hour sputum volume when clinically stable
Pseudomonas growth

23. Pulmonary Function Test

24. BRONCHIECTASIS
diagnosis

25. Diagnosis
Clinical suspicion : chronic symptoms, cough, sputum variable in amount and consistency and content, wheezes and shortness of breath
Physical exam : wheezes, squeaking sounds, variable air entry and added sounds. Clubbing
Chest radiograph : 20 – 40 % patients with mild bronchiectasis may have normal or non specific findings

26. Chest radiograph
Ring opacities or cystic spaces as large as 2 cm in diameter resulting from cystic bronchiectasis, sometimes with air-fluid levels
Cysts related to cystic bronchiectasis, sometimes with fluid levels (demilunes)
Tubular opacities
caused by dilated, fluid-filled bronchi - These may occur as Y or V shapes or as "finger-in-glove" opacities, which radiate from the pulmonary hilum
Increased size and loss of definition of the pulmonary vessels in the affected areas as a result of peribronchial fibrosis
Crowding of pulmonary vascular markings from the associated loss of volume, usually caused by mucous obstruction of the peripheral bronchi
Oligemia as a result of reduction in pulmonary artery perfusion (severe disease)
Signs of compensatory hyperinflation of the unaffected lung

27. Bronchography

28. Tram line
Tram track
Parallel line opacities (tram tracks) caused by dilated, thickened bronchi are seen along their length

29. Radiology – signet ring
The signet ring sign corresponds to a dilated bronchus immediately adjacent to a smaller companion pulmonary artery
Diameter of the bronchus >1.5 of adjacent artery
Lack of tapering

30. HRCT findings
internal bronchial diameter may be greater than that of the adjacent artery, and there may be a lack of bronchial tapering (the same diameter as the parent branch for >2 cm).
bronchial wall thickening may be seen (in 68% of patients).
A cystic cluster of thin-walled cystic spaces may be present, often with air-fluid levels

31. When to suspect CF Cystic fibrosis:
All children and all adults up to the age of 40, presenting with bronchiectasis, should have investigations for cystic fibrosis.
Screening investigations should include both two measurements of sweat chloride, and cystic fibrosis transmembrane regulator (CFTR) genetic mutation analysis
In adults, investigations should also be considered in those with:
Age at presentation >40 years and no other identified cause.
Persistent isolation of S. aureus in the sputum.
Features of malabsorption.
Male primary infertility.
Upper lobe bronchiectasis.
A history of childhood steatorrhoea

32. Yellow nail syndrome Rare disease Lymphedema Pleural effusion
Bronchiectasis in 40%
Chronic sinusitis

33. Guideline in management
bronchiectasis
Guideline in management

34. Mortality M.R. Loebinger et al
91 patients were examined for
Aetiology
Pulmonary function tests
High-resolution computed tomography
Sputum microbiology
Quality of life scores
Follow up over 13 yrs.
Overall, 29.7% of the patients died.

35. Mortality M.R. Loebinger et al
On multivariate analysis
Age
St George’s Respiratory Questionnaire activity score,
Pseudomonas aeruginosa infection
Total lung capacity (TLC), residual volume/TLC and the transfer factor coefficient were
all independently associated with mortality.
In patients with moderate to severe bronchiectasis, mortality is associated with a degree of restrictive and obstructive disease, poor gas transfer and chronic pseudomonas infection

36. End Stage Bronchiectasis
Postbronchodilator FEV1 <30% (especially young females with cystic fibrosis)
Exacerbation of pulmonary disease requiring intensive care unit admission Increasing frequency of exacerbations requiring intravenous antibiotics
Refractory and/or recurrent pneumothorax
Recurrent hemoptysis not controlled by embolization
Other factors to consider: Hypercarbia (PaCO2 >45 mmHg) Rapidly progressive decline in lung function Resting hypoxemia (PaO2 <55 mmHg

37. Worse complications Repeated infections Repeated exacerbations
Lung abscess
Major hemoptysis

38. Guideline in the management
Stop smoking
Influenza and pneumococcal vaccine
Postural drainage
Sputum culture
Stapylococcus
Pseudomonas
Tuberculosis : typical vs. atypical

39. Antibiotics
If there is no previous bacteriology, first-line treatment is amoxicillin 500 mg three times a day or clarithromycin 500 mg twice daily (in patients who are penicillin-allergic) for 14 days.
High-dose oral regimens (eg, amoxicillin 1 g three times a day or amoxicillin 3 g twice daily) may be needed in patients with severe bronchiectasis chronically colonised with H. influenzae.
Ciprofloxacin should be used in patients colonised with P. aeruginosa

40. Antibiotics Long-term oral antibiotics:
Patients having three or more exacerbations per year requiring antibiotic therapy or patients with fewer exacerbations that are causing significant morbidity should be considered for long-term antibiotics. Again, choice will be dictated by sensitivities and local microbiology advice from sputum test results.
Azithromycin is emerging as a useful option for preventing exacerbations in adults and children

41. New trend Nebulizer therapy Immune globulin therapy
Tobramycin
Aztreonam
RDNAse
Immune globulin therapy
Intravenous antitrypsin
Intra bronchial surfactant
Bronchial artery embolization and coiling
VATS segment resection
Thymosin alpha 1

42. SURGERY Evaluate if bronchiectasis is focal, bilateral and diffuse
Must evaluate pulmonary reserve:
PFT
V/Q
Split lung function
Identify syndromes
Evaluate cardiac function

43. Indication for surgery
Failure of medical/conservative therapy
Recurrent respiratory infections
Persistent sputum production
Hemoptysis
Chronic cough
Persistent lung abscess