Pulmonolgy ,it's a common respiratory air way disease with many radiogical features that's vital to learn about it so you can reach the diagnosis easily along with a solid clinical approach

1. BRONCHIECTASIS
Dr. Amira Ahmed

3. TABLE OF CONTENTS
 HIGHLIGHT
 OVERVIEW
 PATHOPHISIOLOGY
 AETIOLOGICAL APPROACH
 CLINICAL APPROACH
 DIAGNOSIS
 COMMON DIFFERENTIAL DIAGNOSES OF BRONCHIECTASIS
 MANAGEMENT
 COMPLICATIONS>>>>>>>>Exacerbation
 REFERANCES

4. HIGHLIGHT
 Bronchiectasis is an end-stage of variety of pathological
processes
Modernization of diagnostic procedures ( CT. scan) and
definition of a clinical picture (repeated lung infections
with a chronic cough and persistent sputum production)
have raised international awareness of the prevalence of
the disease, leading to increasing interest in reviewing
and renewing the treatment guidelines.

5. Suspect bronchiectasis in those with recurrent bacterial
lung infections.
Suspect bronchiectasis in a patient with recurrent episodes
of ‘bronchitis’ prior to presentation, including those with
COPD with Frequent exacerbations and/ or positive sputum
for P. aeruginosa.

6. The diagnostic test of choice for bronchiectasis is
the high resolution CT scan( HRCT)
Sorting bronchiectasis as localized or diffuse
narrows the differential diagnosis.
The underlying cause of bronchiectasis should be
sought after NTM which may complicate pre-
existing bronchiectasis or be the primary cause.

7. OVERVIEW
Bronchiectasis is a chronic suppurative lung disease
characterized by a syndrome of productive cough and
recurrent respiratory infections due to abnormal, irreversibly
dilated thick-walled bronchi.
It usually affects the proximal and medium-sized bronchi with
many etiologies.

8. The management of bronchiectasis can be
challenging because its pathogenic mechanisms is
still evolving.
Its diagnosis and management are particularly more
demanding especially in resource-limited areas.
Patients with disease report worse quality of life
(QOL) than do persons in the general population.

9. In 1950 REID characterized bronchiectasis as Cylindrical the
mild form that shows loss of normal airway tapering and its
the commonest.
Varicose shows more distortion along with more mucus and
sputum production and some bronchi may appear in
beaded form.

10. Cystic or saccular the most severe form in which severe
loss of bronchial wall. structure leads to large balloon-like
dilatations either focal or cystic distortion of the distal
airways.
This type of bronchiectasis often followed severe lung
infections usually in childhood.

12. PATHOPHYSIOLOGY
As consequence of previous lung diseases, especially lung
infections that were not treated sufficiently.
This renders the lung tissue susceptible to further infections,
as the mucociliary defense mechanism fails to regain its full
ability to fight off infectious agents a mechanism further
described as the “vicious cycle theory”

13. Microorganisms produce pigments, proteases, and other
toxins that injure the respiratory epithelium, which induce an
inflammatory response in the host tissue .
The dominant cell types involved in the inflammatory process
are Neutrophils, lymphocytes, and macrophages .

14. Neutrophils are the most abundant cell type in the
bronchial lumen.
 Releasing mediators which destroy the bronchial elastin
and other supporting lung structure .
 Alter the function of the cilial epithelium, leading to
changes in cilia beat frequency and mucous gland
hypersecretion.

15. Weakness of the airways may take many forms. Classic post
infectious bronchiectasis
Pulsion bronchiectasis implies that intense inflammation
originating in the lumen leads to permanent airway dilation as In
ABPA.
Traction bronchiectasis implies that local retractile forces dilate
the airways As in the lung fibrosis

16. A traditional “wet” versus “dry or sicca”
bronchiectasis is now less commonly used
its usually a sequalae of TB and found in the
upper lobes

18. .

20. APPROACH TO DIAGNOSIS OF BRONCHIECTASIS EATIOLOGY
A diverse set of medical conditions is strongly associated with or
complicated by bronchiectasis.
Stduies have reported a lag time between the onset of symptoms and
the formal diagnosis individuals are often diagnosed as
COPD,ASTHMA,TB,CHRONIC SINUS DISEASE.

21. The causes of bronchiectasis are many and varied
Determining the aetiology of the condition may lead to
different management.
IDIOPATHIC cause is around 50% of cases, and this is likely to
be due to an (as yet unidentified) impairment in host defense.
Its lower lobe predominant.

23. Different respiratory infections can cause bronchiectasis, including:
 Pertussis
 Gram negative bacteria (Pseudomonas aeruginosa, Haemophilus
influenzae)
 Viruses (HIV, paramyxovirus, adenovirus, and flu)
 M.Tuberculosis
 Atypical mycobacteria.

24. Bronchiectasis subsequent to infection with Mycobacterium
avium is a typical finding in Lady Windermere syndrome.
Patients suffering from this syndrome are often older,
immunocompetent women without a history of smoking or
previous pulmonary pathologies in the right middle lobe or
lingular lobe.

26. Mounier -Kuhn syndrome(congenital tracheobronchomegaly
due to atrophy of airway elastic fibers)
Williams-Campbell syndrome (absence of cartilaginous rings in
the segmental and sub segmental generations of bronchi).
Weakened airways and airway collapsibility impair the
effectiveness of the cough mechanism.

27. In addition to this schema, determining the etiologic cause of
bronchiectasis may be enhanced by understanding
distribution patterns on HRCT imaging.
The anatomic distribution of bronchiectasis associated with
various etiologies is outlined below

28. FOCAL TYPE:
Is a sequalae of prior infections or endobronchial obstruction
DIFFUSE TYPE:
Most often in patients with genetic ,immunological or anatomical
defects with more expansive list of possible causes

29. Left lung is involved more than right lung more in lower lobe and
lingula. (common focal type)
Lower lobes are involved more than upper lobes due to more
efficient drainage of upper lobes by gravity.
Smaller bronchi with less supportive cartilage are predominantly
involve.

30. Infection Lower lobes, Right middle.
lobe, and the lingula
Obstruction Right middle lobe
Tuberculosis and Upper lobes
chronic fungal
infections
ABPA Upper lobes (central bronchi -proximal BX )

33. ASSOCIATED CONDITIONS:
 Chronic sinusitis
 Yellow nail syndrome
 Marfan's syndrome
 Connective tissue diseases
 Rheumatoid arthritis( up to 35% of pts have bronchiectasis)
 Inflammatory bowel disease.

34. CLINICAL APPROACH
The clinical manifestations result due to pathophysiologic
mechanisms caused by following anatomic alterations:
I. Incomplete obstruction:
Hyperinflation of the distal alveoli as a result of expiratory check-
valve mechanism
I. Complete obstruction:
consolidation , collapse and fibrosis

36. Fatima a33-year-old house wife, lifelong nonsmoker from Kassala city
has symptoms of chronic persistent productive cough on a daily basis
for more than 1 yr. The sputum can be clear or pale yellow, and once
she had haemoptysis , She also complaining of weight loss, tiredness
and SOB provoked by less than 10 min walk.
Prior to the current bout of symptoms, she has been having recurrent
cough for 3 years, she was admitted several times and treated as
pneumonia , when TB work up finally done it was found to be
positive she had received two courses of anti-tuberculosis
medications 1st treatment in 2015 and second treatment in 2017 and
she did not completed 6 months regimen for either.
She denied that she have any other chronic systemic disease.

37. HISTORY
Persistent cough with daily excessive sputum production /foul
smelling sputum.
 Recurrent episodes of upper or lower pulmonary infections
Pleuritic chest pain.
Haemoptesis.
mMRC SCALE grade 3

38. PAST HISTORY
 Childhood symptoms, previous pneumonia/viral illness or
pulmonary TB, gastric aspiration, asthma, joints pain or
deformity , infertility, exposure to smoke
FAMILY HISTORY
 Positive FHx of similar condition.
 Primary ciliary dyskinesia (PCD) ,(Kartagener's syndrome –
sinusitis, situs inversus and bronchiectasis), cystic fibrosis
(CF)

39. CLINICAL ASSESSMENT
GENERAL EXAMINATION
 Coughing (sputum pot)
 ill built (in advanced disease)
 Distended neck veins
 Upper airway ( nasal polyps)
 Breathless at rest ( Use of accessory muscles(
 Barrel chest (increased AP diameter)
 Chest movement (B/L equal / asymmetrical)
 Feature of underlying disease
 Clubbing (2%) ,Tar staining (30% of COPD has NCFB)
 Cyanosis (advanced disease)

40. VITAL SIGNS
Increased
o RR
o PR &BP
o TEMPRETURE
o Oxygen saturation N/

41. CHEST ASSESSMENT FINDINGS-PRIMARILY OBSTRUCTIVE
 Trachea central
 Cricoid-notch distance normal /(reduced )
 Normal chest expansion / (reduced horizontally)
 Normal TVF /(Decreased due to decreased lung density)

42.  Resonant percussion note / ( Hyper resonant)
Vesicular breathing / Diminished breath sounds and
Prolonged expiration.
Widespread expiratory Wheeze
Inspiratory coarse Crackles that alter with coughing
Mid Inspiratory squawks

43. CHEST ASSESSMENT FINDINGS-PRIMARILY RESTRICTIVE
Trachea is midline /deviated to affected side
Chest expansion is reduced in affected area.
Increased TVF (increased lung density ) / reduced.
Dull percussion note
Bronchial breath sounds
Whispering pectoriloquy (Increased lung density)
End inspiratory Crackles that not alter with coughing

44. FURTHER ASSESSMENT :
 LN
 CVS : JVP
Sings of Pulmonary hypertension
Apex location /Apex displacement
TR
Limbs pitting / sacral odema.
ABDOMEN: + Enlarged tender liver

45. DIFFRENTIAL DIAGNOSIS
presence of a productive cough, clubbing, and coarse crackles has
the following differential diagnosis:
 Bronchiectasis
 Lung abscess
 Carcinoma of the lung (Tar staining, lymphadenopathy)
 Pulmonary fibrosis (the crackles are not alter with coughing)

46. COMMON DIFFERENTIAL DIAGNOSIS

47. DIAGNOSIS
Usually made clinically, with HRCT chest for confirmation.
Investigations are aimed at:
 Confirming the diagnosis.
 Functional assessment.
 Identifying a treatable underlying cause for the bronchiectasis (50%)
 To look for comorbidity and complications.
 optimizing management to prevent exacerbations and lung damage

48. INITIAL INVESTIGATIONS
FBC with differential
 Increased hematocrit and hemoglobin
 Hemoglobin may be low (due to anemia of chronic inflammation)
 Increased WBC in acute infection
LFT
RFT
SPUTUM MICROSCOPY Standard microscopy, culture, and
sensitivity(MC&S), acid- fast bacillus (AFB), and fungal cultures
PFTs with reversibility testing
LUNG FUNCTION TEST :
FEV1/ FVC maybe normal/obstructive or restrictive

49. OBSTRUCTIVE:
FEV1 ↓
FVC ↓/N
FEV1: FVC ↓
RESTRICTIVE:
FEV1 N/↓
FVC ↓
FEV1:FVC N/↑

50. FLOW VOLUME LOOP.

51. ABG
Mild to moderate stages:
Acute alveolar hyperventilation with hypoxemia (Respiratory
alkalosis)
o ↑PH
o ↓PaCO2
o ↓PaO2
o N /HCO3

53. ECG:
As a base line to assess QTc and to look for
complications
 Normal (usually)
 Features of Right Ventricular Hypertrophy (RVH) and
Cor pulmonale

54. Typical appearance of RVH:
•Right axis deviation (+150 degrees).
•Dominant R wave in V1 (> 7 mm tall; R/S ratio > 1)
•Dominant S wave in V6 (> 7 mm deep; R/S ratio < 1).
•Right ventricular strain pattern with ST depression and T-wave inversion in V1-4.

55. Diagnostic criteria
•Right axis deviation of +110° or more.
•Dominant R wave in V1 (> 7mm tall or R/S ratio > 1).
•Dominant S wave in V5 or V6 (> 7mm deep or R/S ratio < 1).
•QRS duration < 120ms (i.e. changes not due to RBBB).
Supporting criteria
•Right atrial enlargement (P pulmonale).
•Right ventricular strain pattern = ST depression / T wave inversion
in the right precordial (V1-4) and inferior (II, III, aVF) leads.
•S1 S2 S3 pattern = far right axis deviation with dominant S waves in
leads I, II and III.
•Deep S waves in the lateral leads (I, aVL, V5-V6).

56. ESSANTIAL INVESTIGATIONS
 RADIOLOGY
CXR sensitivity is only 50%
HRCT scan is the golden standard tool its 97% sensitive in
detecting disease (slices <1mm) and (3mm slices) are needed to
exclude a central airway lesion.

57. Tram line sign• Parallel line opacities (dilated, thickened bronchi)

59. Finger in glove -Mucoid impaction)

60. Tree in bud -faint reticulonodular opacities

62. Varicose and cystic bronchiectasis with mucus plugging in upper lobes.

63. Cylindrical BX and tree-in-bud pattern / lower lobes and middle lobe

66. Bunch of grapes sign

69. Tree in bud

73. Mosaic attenuation of the lung due to air trapping in segments of lung affected by
bronchiectasis. The air trapping gives rise to hyperlucent (darker) pulmonary segments.

74. Bronchiectasis (straight arrows), areas of decreased attenuation and
vascularity (curved arrows), and emphysema (small black arrow
heads)

78. A)Chest radiograph showing dextrocardia. B) HRCT cylindrical Bx

81. ADDITIONAL INVESTIGATIONS
CFTR mutation screen and SWEAT TEST.
Autoantibodies (ANA, Rh.F , dsDNA) if associated arthritis/ connective
tissue disease.
Vaccination response to tetanus, H. influenza B, and pneumococcal
antibodies. (e.g. IgG subclass deficiency ) Detailed immunological
investigation (including neutrophil and lymphocyte function studies,
genetic analysis)

82. Nasal nitric oxide +/ - brushingsBIOPSY(in tertiary centre) to assess
ciliary beat frequency with video microscopy to exclude PCD in those
with risk factors.
A1AT levels if deficiency suspected.
Barium swallow/ esophageal imaging if recurrent aspiration
suspected.
Vasculitis screen (RF, ANCA, ANA, ENA, and anti- CCP antibodies) if
connective disease/ arthritis/ vasculitis associated bronchiectasis

83. Immunoglobulins A, M, G
Aspergillus precipitins, Aspergillus- specific radioallergosorbent test
(RAST), total IgE.
 HIV serology.
Bronchoscopy to exclude a foreign body if suggested by CT obtain
microbiological samples if unusual clinical presentation or failure to
respond to standard antibiotics
 Echocardiography.

84. MANAGEMENT
There is no cure for bronchiectasis, but it can be treated. Watch
for early warning signs of a flare-up and work with a health care
provider to find the best treatment plan.
In a few rare cases, surgery to remove damaged lung tissue in
only one area of the lung, may be curative.
Research and clinical trials are taking place now to find better
treatments and cure(s) for bronchiectasis.

85. Non cystic fibrosis bronchiectasis severity score: The
FACED score

87. GENERAL MANAGEMENT OF NON .CF.B
The main aims of management of NCFB are:
 Ensure the patient understanding.
 Treatment of any underlying medical condition and prevent its
progression.
 Prevention of exacerbations and progression of the disease.
MEASURES:
 Smoking cessation.
 Optimizing hydration status .
 Optimize nutrition, treat weight loss or low body mass index
aggressively as this is associated with a poorer prognosis

88.  Daily chest physiotherapy ,postural or autogenic drainage
Exercise regimes— important to prevent general deconditioning.
Nebulized saline hypertonic saline, Nebulized DNase may improve
airway clearance.
B2agonist enhance airway clearance
Acetylcysteine may reduce sputum viscosity. If required (6- month).
 Cough augmentation— using flutter valves/ positive pressure
devices

90. Antimicrobial chemotherapy Reduction of bacterial load and
prevention of 2° airway inflammation and damage.
Oxygen therapy if in respiratory failure.
NIV as nocturnal / used as a bridge to transplantation.
LTOT use the same criteria as for COPD .
Steroid if indicated >>In the context of pre existing bronchial
asthma or COPD. >> ABPA
Remember There is no indication for inhaled steroids in isolated
bronchiectasis.

91. Refer for surgery in rare cases, for localized resection of affected area.

92. Refer for lung transplantation if indicated (more in CFB) :
 Young less than 20yrs female with rapid deterioration
 Severe haemoptysis despite embolization
 Recurrent pneumothorax
 An exacerbation with inpatient admission to RICU or HDU
 Oxygen dependent respiratory failure , type 2.Rf and PHT
 FEV1 ≤30 % predicted or FEV1 >30 with rapid progressive
deterioration.

93. FURTHER MANAGEMENT
Self- management plan Patients need an individual plan for
exacerbations , which usually involves having a supply of home
antibiotics.
Annual influenza / pneumococcal vaccinations.
 Osteoporosis prophylaxis (if on long- term steroids).
Refer for pulmonary rehabilitation if breathlessness limits activities
of daily living

94. Reflux treatment if aspiration
Associated rhinosinusitis is seen in up to 70%. Treat with nasal
steroid; consider antibiotics if infection likely.
Immunoglobulin replacement therapy Patients found to have
immunoglobulin deficiency should be referred to an
immunologist.

95. ASSESSMENT OF DISEASE RESPONSE:
Decreased in sputum volume and change to a mucoid
Improvement in systemic symptoms
CRP
Spirometry and PFT

97. COMPLICATIONS
 Acute Infective exacerbation.
 Recurrent pneumonia
 Empyema
 Lung abscess
 Septic emboli( cerebral abscess)
 NTM infection
 ABPA.
 Haemoptysis : Massive hemoptysis (usually from tortuous
bronchial arteries around damaged lung is a life- threatening
emergency)

98. Pneumothorax.
Lung collapse.
Respiratory failure.
Cardiovascular disease—the rate correlates with severity
of disease and frequency of exacerbations.
Amyloidosis.

99. AN EXACERBATION is usually a clinical diagnosis, with an increase
in sputum volume and tenacity and with discoloration.
It may be associated with chest pain, haemoptysis and wheeze, and
systemic upset— fevers, lethargy, and anorexia.
The CRP is not always elevated
The deterioration in three or more of the following key symptoms for
at least 48 hours.

100. Recognize an acute exacerbation with 4 out of 9 criteria:
1.Change in sputum production
2.Increased dyspnea
3.Increased cough
4.Fever
5.Increased wheezing
6.Malaise, fatigue, lethargy
7.Reduced pulmonary function
8.Radiographic changes
9.Changes in chest sounds

101. COMMON MICRO ORGANISMS
H.Influenzae
 Staph .aureus
Streptococcus pnemoniae
P.Aeruginosa which found in patient with frequent exacerbation
worst CT appearance and faster decline in PFT.
Remember in stable pt. with frequent exacerbation>>P.Aeruginosa

103. MILD DISEASE WITH EXACERPATION
 Sputum sample for M.C and S prior to antibiotic.
 Empirical antibiotic initially then tailored to the colonizing
organism.
 No prior positive microbiology:
Amox 500mg-1 g TDS or /
Doxycycline 100mg OD
Duration 10-14 days

104. EARLY RELAPSE:
Defined as sputum turned purulent with in 6-8 wks.
Plan:
 Reassess the pathogens by sputum c/s.
 Long course antibiotics: PO. AMOX 500 mg BD
PO.DOXYCYCLINE 100mg OD
Duration 6 wks.
TREATMENT FAILURE
 Change to iv antibiotics until clinical improving .
 long term prophylactic antibiotics. Start 2 days after sputum has
cleared often for 2wks - 6 months
 And check sputum if purulent switch to iv abx.

105. SEVERE DISEASE WITH EXACERBRATION
 Hospital Admission.
 Sputum for c/s prior to antibiotics.
 IV.Abx often for 2 wks. / 2days after the sputum has cleared.
 Assess treatment response by fall in sputum volume / change
to mucoid/improvement in systemic symptoms and CRP.
 Planned for regular cyclical IV.Abx.

106.  Frequent exacerbations need Long term management
with Combination of Macrolides plus Nebulized colistin or
gentamicin.

107. First Isolated P .aeruginosa
 Oral antipseudomonal Antibiotics for 2 wks.
 Sputum culture if still positive give IV for 2 wks. plus nebulized
antibiotics (Colistin /gentamicin/tobramycin) for 3 months.
 If there's no response give combination IV.Abx antipseudomonal
and aminoglycosides.

109.  STATINS might alter neutrophil apoptosis. in patients with
bronchiectasis without Pseudomonas infection showed there
were clinically meaningful improvements over 6 months with
an:
 Increase in the number of apoptotic neutrophils in sputum.
 Decrease in the total number of neutrophils in sputum
 Reduction in levels of interleukin-8 in serum.
NEW THERAPIES AND NEW PRESPECTIVES

110.  BRENSOCATIB is an oral reversible inhibitor of
dipeptidyl peptidase 1 (DPP-1), an enzyme responsible for
the activation of neutrophil serine proteases.
Reduction of neutrophil serine protease activity with
Brensocatib in patients with bronchiectasis was associated
with improvements in bronchiectasis clinical outcomes.

111. .

112. REFERANCES
1. Murray & nadels textbook of respiratory medicine
2. Oxford handbook of respiratory medicine
3. OST
4. Up to date .Com
5. https://www.ncbi.nlm.nih.gov/books
6. British Thoracic Society Guideline for bronchiectasis in adults | Thorax (bmj.com)
7. Epomedicine . Bronchiectasis Revision Notes [Internet]. Epomedicine ; 2016 Feb 12 [cited 2022 Apr
5]. Available from: https://epomedicine.com/medical-students/bronchiectasis/.
8. https://litfl.com/right-ventricular-hypertrophy-rvh-ecg-library
9.Bronchiectasis: a case-based approach to investigation and management - PubMed (nih.gov)