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Bronchiectasis Drkhinchi 121027215553 Phpapp02
Bronchiectasis Drkhinchi 121027215553 Phpapp02
Airway Destruction +
Airway Dilation
FIGURE 2
CF vs. Idiopathic Bronchiectasis
CYSTIC IDIOPATHIC
FIBROSIS BRONCHIECTATIS
Rheumatoid arthritis
Systemic lupus erythematosus
Relapsing polychondritis
Inflammatory bowel disease -
Ulcerative colitis and Crohns
disease
Evidence for dysregulated immunity in
bronchiectasis
Increased susceptibility to infection - bacterial, non-
tuberculous mycobacterial (NTM), and aspergillus-related
lung disease
Associated with autoimmune disease such as the
inflammatory bowel disease, ulcerative colitis
Neutrophils are markedly raised, as predicted from high local
levels IL-8
Associated with immune deficiency syndromes such as TAP
deficiency syndrome
Suppurative Lung Disease
Bronchiectasis
Lung abcess
Empyema
Brochiectasis
Def:destructive lung disease :chronic permanent and
abnormal dilatation of bronchial wall with variable
inflammatory process in the lung. Chronic bronchial
sepsis
Pathology: non-inflammatory:congenital
acquired:
Bronchial wall dilatation.
excess mucus.
loss of connective tissue support
inflammation with metaplasia of ciliated
epithelium to squamous or columnar + micro-abcess formation.
Collapsibility of the wall leads to obstructive defect on spirometry.
Notes
Bronchiectasis is of two types:
1- Dry: there is dilatation, destruction,
but no purulent secretion.
2- Suppurative (refers to chronic
bronchial sepsis): this is
bronchiectasis that have suppuration
( pus formation).
Pathogenesis
Vicious cycle:
infectious insult & impaired drainage &/or defect in host defence
Role of positive :
genetic susceptibilty
family history
Notes
Normally, in healthy individuals the immune system and
mechanical system (cilia function) are intact, resulting in
good clearance of microorganisms leading to recovery.
Therefore, with impaired cilia function and/or host defense
a vicious cycle (ongoing inflammation) will occur leading to
bronchiectasis.
People with family history and genetic susceptibility (e.g a-
1 antitrypsin deficiency), when having a microbial insult,
they can not clear it probably because of defective immune
and/or mechanical drainage system.
The most important organisms are: H.influenza,
encapsulated streptococcus pneumoniae, and
pseudomonus.
Classification
Spectrum:
1.follicular cylindrical:transmural inflammation, loss of bronchial
elastic tissue, mucosal edema; post infectious
2.Saccular:ulceration ,craters; general dilatation specially terminal
(saccules).
(varicose:distortion by scarring and obstructions)
3. Atelactatic:
localised, could be secondary to 1, or 2.importance to site and
distribution: lobar or segmental.
Notes
Another type of classification is:
1- follicular cylindrical: it is called follicular
because of presence of lymphoid follicles in the
bronchus. But it is generally called cylindrical
because it is transmural and localized to one
bronchus.
2- saccular: so called because there are succule
like formation at the terminal part of the
bronchus.
- varicose: this is when you have two types
together cylindrical and saccular.
Causes of bronchiectasis
*Immune deficiency
Primary: children: hypogammaglobinaemia:repeated
sinopulmonary infection
Secondary: AIDS, CA
Contd Causes
*Mucociliary clearance defects
-Genetic
-Primary ciliary dyskinesia :cilia abnormal
50% Kartegners:S.I, sinusitis Bronchiectasis.
-Cystic Fibrosis: abnormal mucus content.
*Acquired: -Youngs: sinusitis ,bronchiectasis ,obstructive azoospermia.
Abnormal mucus.
- -Post-infective bronchial damage:measles, pertussis.
MAI.Viral.Mycoplasma
- Toxins: SO2,smoking, lidocaine. Asthma.
*Rheumatic and systemic inflammatory diseases:e.g IBD (inflammatory
bowel disease).
*Idiopathic (including diffuse panbronchiolitis), good % with no cause
Notes
In cystic fibrosis, the cilia is normal but the mucus is
abnormal because there is a block in the chlorine
channel (not responsive to cyclic AMP) causing the
mucus to become more sticky. This disease is
characterized by increased Na and Cl in sweat, which is
detected by sweating test.
Kartegner syndrome: is a type of primary ciliary
dyskinesia which is also associated with sinusitis,
bronchiectasis, and transposition of the viscera.
Youngs disease is characterized by high lipid content of
mucus.
Idiopathic: panbronchiolitis (responsive to macrolide) is
found mainly in Japanese and East Asia.
Conts Notes
ABPA causes hyper immune activity.
Atypical mycobacterial infection and
uncontrolled asthma can cause
abnormal cilia.
*Mucociliary clearance defects
*Genetic: Cystic fibrosis: AR, Gene
mutation,chromosome 7,Transmembrane conductance
regulator pr-CFTR
commonest:deletion at 508
impairment of chloride channel.non respondent cyclic-
AMP. Cl- and water not excreted but Na and its water
absorbed ,mucus get thickened.Sweat content of Na , Cl
altered.
Clinical manifestation of cystic fibrosis
Primary
Bronchiectasis *Nasal polyps
Bronchiolitis, bronchitis * Cirrhosis
Infertility * Meconium ileus
Pancreatic insufficiency
Pneumonia
Salt loss
Secondary
*Atelectasis * Allergic bronchopulmonary
*Clubbing aspergillosis
*Malabsorption * cholelithiasis
*Heat prostration * Cor pulmonale
*Hypochloraemic * Conductive hearing loss
hypokalaemic alkalosis * Diabetes mellitus
Diagnosis of Bronchiectasis
1.History: usually :muco-purulent sputum, cough.
occasionally recurrent hemoptysis, recurrent fever
Less specific : dyspnea, wheeze ,pleuritic pain
2.Examination: Early: normal
nowadays:clubbing 3%,crackles70%
rarely :Core pulmonale( right ventricular
hypertrophy due to pulmonary hypertension),CCF.
Notes
The most important sign is is abundant productive cough
increasing with lying down.
The second important manifestations are lung abcess,
infective emboli, clubbing and amyloidosis (AA type).
How hemoptysis occurs?
An insult causes injury, followed by healing and
neovascularization. These new formed blood vessels are
weak and fragile, hence easily ruptured causing
hemoptysis.
- Wheeze: musical sounds produced by air passing through
narrowed airways (during inspiration & expiration).
- Crackles (crepitations): non-musical sounds mainly heard
during inspiration caused by reopening of occluded small
airways.
Investigation
Sputum: gram stain,selective media ( it is done not to
miss certain organisms),semi quantitative
!!TB, fungal
CXR: early volume loss,vascular crowdening
late:cylindrical tramline,cystic.
Disribution: central ABPA,
LL: idiopathic
UL: CF or variant
Investigation
Bronchogram: surgery
HRCT: standard
Sinus X-Ray
PFT: normal,obstructive,air trapping
Specific: Ig level:IgA (in immunocompromised
patients).
precipitin level is measured in skin, sputum and serum. If it
is high in 2 out of the 3 sites, we think of ABPA.
Brochoscopy
Notes
Bronchogram: do a bronchoscope then inject a
dye. It is done when you are planning for
surgery.
HRCT= high resolution CT scan.
Sinus X-ray is done to rule out sinusitis
PFT is early normal, later:obstruction/ air
trappin
Bronchoscopy is indicated in case of hemoptysis
and it is important to rule out obstructive
lesions (e.g. foreign bodies) and TB. These
three conditions are not responding to
antibiotics.
Notes
The previous slide shows CT taken
during inspiration.
Usually, a blood vessel has a
bronchus beside it that has the same
size.
So, if you see a bronchus larger than
the accompanied vessel then suspect
bronchiectasis.
Notes
The previous CT was taken for the
same patient during full expiration,
which is better than the full
inspiratory CT.
Contd investigation
UGI Endoscopy
Anti protease level
Sweat test
Genetic studies
Rh. Factor
Complication
Mostly: infective
exacerbation,haemoptysis
Rarely now: metastatic spread of
infection empyema
amyloidosis clubbing
joint pain
Note: certain complications are rare because of early
detection.
Management
Prevention:
Medical treatment:control infection ,bronchial hygiene
Antibiotic:acute exacerbation: cover I.infl., strep.
Prophylactic:several protocols: commoner :
10days/Month or 10 days alternate with 10 free days.
Bronchodilator
IV Ig if deficient
Oral Steroid:only ABPA vs inhaled steroids
Mucolyte :debatable.role of ACC ( N-acetylcystin
which is antioxidant), DNAs
Anti-fungal :itracanazole: ABPA
Future treatment:Antiprotease replacement
CF treatment (very complicated)
Notes
Management of bronchiectasis includes:
- 1- Giving Igs for immunocompromised patients.
- 2- Vaccination for H. influenza, pneumococcus and
influenza.
- 3- Screening for TB (very important).
- 4- Bronchodilators (almost always given).
- 5- Steroids (only for ABPA because of the
hyperimmunity).
- 6- mucolytics: make sputum more liquid and less viscus
(not important except DNAs in cystic fibrosis patients to
improve their lung function).
Contd management
Physiotherapy
Corner stone:Postural drainage,
hydration ,nebulized saline
*Surgery:
.Massive haemoptysis: > 600ml/day.
.Localized troublesome resectable bronchiectasis
.Palliative for important stump.
Notes
- physiotherapy: teach the patient how to drain his lung
(lungs should be drained daily).
- Surgery: is indicated:
1- when there is severe hemoptysis
2- if one lobe causing problems to the patient, so you
resect it to prevent damage to other lobes
3- if you have a diffuse lung disease and one particular area
which is more problematic, then you resect it.
- Mild hemoptysis: loss of 200-300ml/day.
- Moderate hemoptysis: loss of 300-600ml/day.
- Severe hemoptysis: loss of > 600ml/day.
Contd management
Haemoptysis:
*Mild: antibiotic
*Severe: surgery or bronchial artery embolization
Lung Transplant: always double.
- Indication for transplantation: bilateral, advanced, and
bleeding bronchiectasis.
- 2 lungs should be transplanted because if you transplant
one lung only, the diseased lung you left in the body can
affect the new transplanted lung to become also
infected.
Bronchiectasis
Chronic dilation of the bronchi marked by
fetid breath and paroxysmal coughing, with
the expectoration of mucopurulent
matter.
Morphological types
Cylindrical or tubular bronchiectasis
Varicose
Infection
Inflammation
Bronchiectasis
Altered development
Inflammation
Inappropriate inflammation
e.g. ABPM, CF (?)
Oxidant stress
-low antioxidants associated with worse disease
-dysregulation of signaling and cellular function
Childhood Infections M. Tb.
Infection
Bronchiectasis
Altered development
Impaired Clearance
Altered ciliary Function
PCD, smoking, CFTR, Youngs
Mucus rheology
CFTR, Mucoid Ps. A
Acquired
Chemo-immunomodulation
Congenital/innate
HIV/AIDS Chemo-Immunosuppression
anti-TNF, MTX, anti-neoplastics
Infection
Bronchiectasis
Altered development
HIV/AIDS Chemo-Immunosuppression
Infection
Bronchiectasis
Altered development
HIV/AIDS Chemo-Immunosuppression
Infection
Bronchiectasis
Altered development
HIV/AIDS Chemo-Immunosuppression
Infection
Bronchiectasis
Altered development
Bronchiectasis
Epidemiology and Etiology
Who?
Patients - with altered immune system
- with persistent respiratory symptoms
Why?
Persistent inflammation in the respiratory system
Bronchiectasis Therapy
Decrease inflammation
antibiotics
clearance
Flutter, IPPV, Vest, Bronchodilators, hypertonic saline
(anti-inflammatory chemotherapy)
Steroids, macrolides, interferon-gamma, ibuprofen
(surgical resection)
When to suspect bronchiectasis?
Focal Systemic
Sputum production Malnutriton/wasting
Mild <15 cc/d Chronic Inflammation
Moderate 15-150 gammaglobulinemia
cc/d CRP
Sed rate
Severe >150 cc/d
anemia
Hemoptysis
Dyspnea
Chest pain
Bronchiectasis Therapy
Antibiotics
Yes.
Con
Pro Select resistance
Decrease inflammation Cost
Side effects
Slow progression adherence difficult (e.g.
Eradication? Huong et al.)
Bronchiectasis Therapy
Antibiotics
Con
Pro Select resistance
Decrease inflammation Cost
Side effects
Slow progression adherence difficult (e.g.
Eradication? Huong et al.)
Bronchiectasis Therapy
Antibiotics when to use?
No:
Yes: Minimal disease without
Evidence of organism
Patient Intolerant
exacerbation
Unaffordable
Progressive decline
Frequent
exacerbator
Active inflammation
(?)
Bronchiectasis Therapy
Decrease inflammation
Clearance
Immunomodulatory chemotherapy
proposed therapies:
Steroids
Macrolides, tetracyclines
interferon-gamma
ibuprofen
Bronchopulmonary Hygiene
removal of respiratory secretions is beneficial
chest percussion and postural drainage
chest clapping or cupping
inflatable vests or mechanical vibrators
Oral devices that apply positive end-
expiratory pressure maintain the patency of
the airway during exhalation
Maintaining adequate systemic hydration,
enhanced by nebulization with saline,
Acetylcysteine delivered by nebulizer thins
secretions
aerosolized recombinant human DNase
(rhDNase) in patients with cystic fibrosis
Surgery
Localised bronchiectasis
Proximal obstructive lesion
Massive hemoptysis
Recurrent infections
Vicious loops
Infection
Bronchial
Obstruction Inflammation
Bronchiectasis
Summary
diagnosis management
History Treat bronchiectasis
Prior infections, exposures Clearance
Time course Antibiotics
Other manifestations? Immune-modulation
Chest Imaging Balance burden of disease vs
Define region, pattern burden of therapy
Sputum culture (Sputum, Symptoms, PFTs, Weight, X-rays)
Determine causal disease Other
sweat testing Underlying disease therapy
immune testing
serologic testing Transplantation
Management of complications
Collapse, plugs, hemoptysis
Antibiotics
Vicious loop tobramycin
Infection
Bronchial
Obstruction Inflammation
Clearance Anti-inflammatory
Albuterol, DNase, Inhaled steroids
Therapy vest
Bronchiectasis
Bronchiectasis
Clinical:
Cough (90 %)
Daily sputum production (76%)
Dyspnea (72%)
Hemoptysis (56%)
Recurrent pleurisy
Pathophysiology
2 Prerequisites:
Infectious insult
Airway obstruction:
intraluminal tumor, foreign body, lymph nodes, COPD
Immunodeficiency:
ciliary dyskinesia, HIV, hypogammaglobulinemia, cystic fibrosis
(obstruction and immunodef.)
Characteristic central bronchiectasis 2/2 ABPA
Note characteristic location in the upper lobes and superior segments of
lower lobes
Exacerbation: Etiology +Rx
Colonization/infection:
Hemophilus
Pseudomonas
MAI
Aspergillus
Treatment:
fluoroquinolone
Prevention
Antibiotics-Controversial:
Consider Macrolide TIW
Cipro qd X 7-14 D/ month
A
B
E
Circle filled
with air
SEVERE BRONCHIECTASIS
RINGS (CYSTS) CONTAINING AIR-FLUID LEVELS
BRONCHIECTASIS
THE CT SCAN
Signet ring sign
Tram-tracks
String of beads
Circles filled with air or air and fluid
Tubular and branching opacities
Bronchi visible within 1 cm of the pleura
Scarring
Normal pulmonary
artery (pearl)
Dilated bronchus
(ring)
SIGNET-RING SIGN
Dilated bronchus
BRONCHIECTASIS
Bronchi visible
within 1 cm of
the pleura
String
of beads
BRONCHIECTASIS
Destroyed
lung
(Scarring)
BRONCHIECTASIS
CAUSES OF BRONCHIECTASIS
Congenital
Acquired
CONGENITAL CAUSES OF
BRONCHIECTASIS
Cystic fibrosis
Immotile cilia syndrome
CYSTIC FIBROSIS
Diffuse bronchiectasis
Most severe in the upper lobes
CYSTIC FIBROSIS
Worse in the
upper lung
zones
CYSTIC FIBROSIS
IMMOTILE CILIA SYNDROME
Diffuse bronchiectasis
May have situs inversus (Kartageners
syndrome
Bronchiectasis
Dextrocardia
KARTAGENERS SYNDROME
Bronchiectasis
KARTAGENERS SYNDROME
KARTAGENERS SYNDROME
KARTAGENERS SYNDROME
Dextrocardia
Dilated
bronchus
CAUSES OF ACQUIRED
BRONCHIECTASIS
Post-infectious
Post-obstructive
aspirated foreign body
slow-growing tumour
POST-INFECTIOUS
BRONCHIECTASIS
Affects the part of the lung which was
involved with pneumonia
Often diffuse as most commonly secondary to
a viral pneumonia
POST-OBSTRUCTIVE
BRONCHIECTASIS
Focal or localized because only distal to the
obstructing lesion
Dilated bronchi distal to obstruction filled
with mucus instead of air
FOCAL BRONCHIECTASIS
FOCAL BRONCHIECTASIS
Branching
tubular opacities
FOCAL
BRONCHIECTASIS
Focal bronchiectasis
due to slow-growing
endobronchial tumour
Focal bronchiectasis
due to slow-growing
endobronchial tumour
Dilated bronchi
filled with mucus
Branching
tubular opacity
Dilated bronchi
filled with mucus
instead of air due to
proximal obstruction
PULMONARY EMBOLISM
RADIOLOGICAL FEATURES
Lung - Pathology
Pulmonary edema
Distended peripheral
bronchi (Due to weakening
of wall)
Bronchiectasis
Gross
PRIMARY ANTIBODY
DEFICIENCY
(PAD)
&
BRONCHIECTASIS
(UKPIN / BTS GUIDELINES)
BRONCHIECTASIS
A destructive lung disease characterised by:
Neutrophil proteases
(acute infection in a normal or compromised host)
Epithelial injury
+
Structural protein damage
Damaged, dilated airway
Mucous retention / chronic, recurrent infection
Ongoing inflammation / tissue damage / repair
BRONCHIECTASIS - aetiology
Infection
- pertussis, influenza, measles, TB, necrotising peumonia
Bronchial obstruction
- mucoid impaction, ABPA
Congenital anatomical lung abnormality
Inherited disorders
- ciliary dysfunction
- cystic fibrosis
- alpha-1 AT deficiency
Undefined (29 - 49%)
BRONCHIECTASIS OF UNDEFINED
REF.
AETIOLOGY NOS. ANALYTE % age ABN.
ASSOCIATION n %
Idiopathic 80 53
ABPA 11 7
PAD 11 7
Neutrophil defect 1 <1
Rheumatoid disease 4 3
Ulcerative colitis 2 <1
Ciliary dysfunction 3 1.5
Youngs syndrome 5 3
Cystic fibrosis 4 3
Post-infectious 44 9
Aspiration/reflux 6 4
Other defineable 2 <1
BRONCHIECTASIS in PAD
CVID
53% (Hausser et al 1983)
44% (Watts et al 1986)
18% (Hermazewski & Webster 1993)
20% (UK PAD Audit 1993-96)
27% (chronic lung disease) (Cunningham Rundles 1999)
58% (Garcia 2001)
43% (Busse et al 2002)
XLA
7% (Hermazewski & Webster 1993)
12% (UK PAD Audit 1993-96)
20% (Quartier et al 1999)
RESPIRATORY INFECTIONS
and/or
- vessel crowding
- loss of vessel markings
- tramline/ring shadows
- cystic lesions/ air-fluid levels
- evidence of TB
Poor:
diagnostic sensitivity
monitoring of progression
3
RADIOLOGY - HRCT
- bronchial dilatation
- bronchial wall thickening
- classification (pathology)
ANTIBIOTICS
Effectiveness established in exacerbations (bronchiectasis) 2
Higher doses for longer periods 4
Local treatment protocols 4
ANTIBIOTIC PROPHYLAXIS
Chronic bronchitis - no place in routine treatment
(Cochrane Database of Systematic Reviews. 3, 2003)