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Outline
Introduc4on
Epidemiology
Type
Clinical
Pathophysiology
Natural
history
Diagnosis
management
Bronchiectasis dene by dilata4on or ectasia of the airways or bronchus Share clinical feature with COPD : inamed and easily collapsible airways, obstruc4ve out ow, recurrent or refractory infec4on , hemoptysis
Epidemiology
No
systema4c
data
on
the
incidence
or
prevalence
for
bronchiectasis
Decline
rate
of
Brochiectasis
aFer
20th
century
due
to
vaccine
and
ATB
development
Es4mate
110,000
individual
in
USA
Prevalence increase by age USA 4.2/100,000 18-34 yr. 272/100,000 72 yr. More common in women USA : increase number of brochiectasis cases associtated with TB or NTM Auckland NZL 1/6,000 admit 16.4/100,000
Type
of
bronchiectasis
Local
lobe
or
segment
Intrabronchial
obstruc4on:
FB
,
slow
growing
tumor
Extrabronchial
obstruc4on
Lymph
node
compression
Diuse
Ciliated
epithelium
dysfunc4on
:
primary
ciliary
dyskinesia
Immunodeciency
:
HIV
,
congenital
and
acquired
hypogammaglobulinemia
Congenital
disorder:
Cys4c
brosis
(CF),
alpha-1
an4trypsin
deciency,
tracheobronchomegaly (
Mounier-Kuhn
syndrome),
car4lage
deciency (
William-Cambell
syndrome
,
young
s
syndrome,
Marfan
sydrome
Rheuma4c disease : RA, inamatory bowel disease, SLE, Sjogren s disease Post infec4on : adenovirus, measle, inuenza
Clinical
nding
Classic
clinical
:
cough
and
the
daily
produc4on
of
mucopurulent
and
tenacious
sputum
las4ng
month
or
year
Complaint
include:
dyspnea,
wheezing,
pleuri4c
pain,
hemoptysis
Clinical
nding
Physical
nding
Crackle
,
wheezing,
were
common
Digital
clubbing
only
2
percent
Urinary incon4nence in the pa4ents with bronchiectasis 47% (10% in general popula4on)
bronchiectasis e4ology Role of infec4on Predominant organism in sputum Airow obstruc4on and hyperresponsiveness Finding of Chest immage Quality of sputum Infec4on, genene4c ,immune defect primary H. inuenza, Pseudomonas aerugenosa prensent Airway dila4on ad thickening , mucoid plug Purulent,
COPD Cigarehed smoking Secondary Strep. Pneumoniae H. inuenza Present Hyperlucency,hyperina4on , airway dilata4on Mucoid, clear
Pathogenesis
Various
mechanism
to
produce
permanent,
pathologic
dilata4on
and
damage
of
the
airways.
Trac4on
brochiectasis
Pulsion
bronchiectasis
Weakness
of
the
airwary
Pathogenesis
Trac4on
brochiectasis Distending
force
of
the
nega4ve
intrapleural
pressure
are
transmihed
to
the
airways.
Fibro4c
lung
change
such
as
sarcoidosis,
inters44al
lung
disorder,
infec4on
such
as
TB,
Local
trac4le
result
xed
dilata4on.
Pathogenesis
pulsion
brochiectasis
Allergic
bronchopulmonary
aspergillosis (ABPA)
is
prototype
Reac4on
to
inhaled
Aspergillus.
Fungi
,
mucoid,
iname
mediator
obstruct
airway
Pressure
distend
the
central
air
way
infec4on
Impaired drainage
Airway obstruc4on
Impair secre4on drainge obstruc4on, impair mucociliary, Collec4on of sputum and bacteria increase infec4on rate Airways obstruc4on, dilate bronchus , thickening bronchial wall Increase mucus secre4on
Microbiology
Nonenteric
gram-nega4ve
bacteria
commonly
infect
1/3
pa4ent
are
chronically
colonized
with
Pseudoaeruginosa
Staph.
Aureus
raises
the
suspicious
for
Cys4c
brosis
E4ology
cause
of
bronchiectasis
may
be
dicult.
Even
with
exhaus4ve
clinical,
laboratory,
and
pathologic
tes4ng,
up
to
50
to
80%
of
cases
of
bronchiectasis
may
s4ll
be
idiopathic
Lady Windermere syndrome: Primary Mycobacterium avium complex infec4on has been recognized in white women over 60 years of age. Chronic, unrelen4ng cough and middle-lobe involvement. AIDS : repeated respiratory tract infec4ons and impaired host response. Previous pyogenic, pneumocys4c, and mycobacterial infec4ons
AIDS : repeated respiratory tract infec4ons and impaired host response. Previous pyogenic, pneumocys4c, and mycobacterial infec4ons
Orther abnormality: o44s media, GEReux, Infer4le Inves4gate : chromosome iden4cal 5p15- p14,DNA H5
Cy4c
brosis
Abnormal
gene:Cys4c
Fibrosis
Transmembrane
conductane
regulator(CFTR)
decrease
Cl-
secre4on,
increase
Na
reabsorp.
Mucus
plug
airways
obstruc4on.
onset
in
adulthood
of
repeated
respiratory
tract
infec4ons
in
the
absence
of
exocrine
pancrea4c
insuciency,
sinunsi4s
is
recognized
as
a
presenta4on
of
cys4c
brosis
Upper-lobe inltra4on apparent on imaging of the chest and the growth of S. aureus or mucoid Pseudomonas aeruginosa on sputum
Incidence 1:2,000-3,000 west Thai report 1 case Elevated chloride concentra4ons(>55-60 mmol/L) on sweat chloride tes4ng are diagnos4c HRCT-bronchiectasis predominent upper lobe
Disorder
of
immunity
Deciencies
of
IgG,
IgM,
and
IgA
are
risk
for
recurrent
suppura4ve
sinopulmonary
infec4ons
and
bronchiectasis
Acquire
hypogammaglobulinemia
is
the
most
frequent
syndrome
recognized
in
this
group
of
disease
Hx
childhood
with
repeated
sinopulmonary
infec4on
Recurrent lung infec4on with encapsulated capsule S.pneumonia, H.inuenzae. S.aureus, P.seudoaerugenosa The diagnos4c : low level of gamma globulin, failure response to produce an4body following vaccine. Reple4on with gamma globulin can prevent further respiratory tract infec4on and lung damage
Alpha1-an4trypsin
anomaly
A
review
of
the
clinical
manifesta4ons
and
chest
CT
scans
of
pa4ents
with
alpha-1
an4trypsin
deciency
found
that
radiographic
abnormali4es
sugges4ve
of
bronchiectasis
(95
percent)
ATS/ERS
recommend
alpha-1
tes4ng
in
BXSIS
pt.
and
no
other
e4ology
HRCT-severe
diuse
cys4c
bronchiectasis,
emphysema
Bronchial
car4lage
Mounier-Kuhn
syndrome
(
congenital
tracheobronchomegaly
Rare
disorder
associate
:dilate
of
C
ring
of
trachea
bronchi
segment
Underlying
defect
is
absence
elas4c
ber
and
smooth
m.
of
large
airway.
Recurent lower respira4on infec4on in early year and 4 cascade Diagnosis : extraordinary dilata4on of the trachea and central bronchi on CT. Management: consider PEEP support and silicone or metallic stent Lung transplant is an op4on
HRCT may help diag up to 30% Five yr. Follow up : pt. With bronchiectasis and RA were die 5 4mes with RA alone
Cigarehe
smoking
Causal
Role
of
cigarehe
smoking
in
bronchiectasis
is
not
conclusive
Smoking
may
worsen
PFT,
and
progress
of
disease
Young
syndrome
Syndrome
exhibit.
:
Bronchi4s
,
sinusi4s
,pancrea4c
insuciency
like
CF,
obstruc4ve
azoospermia.
Normal
sweat
chloride
levels.
Natural
history
Clinical
course
is
variable
Frequent
1.5
exacerba4ons
per
yr.
US.,
EU,
Ireland
Decline
FEV1
50ml/yr
Accelera4on
decline
Lung
func4on
associate
with
Pseudomonas
aeruginosa
Diagnosis
Inves4ga4on
Aim
Diagnose
bronchiectasis
Evaluate
for
poten4al
underlying
cause
Rx
treatable
cause
Diagnosis
Chest
X-
ray
Abnormal
in
most
pa4ent(
90%)
but
not
specic
picture
Finding
suggest
BXSIS
Tram
track
Mul4ple
ring
shadow
HRCT
scan
There
are
suspicious
clinical
ndings
but
a
rela4vely
normal
chest
radiograph.
The
chest
radiograph
has
abnormal
ndings
and
bronchiectasis
is
strongly
suspected.
Management decisions must be made that depend upon the extent of bronchiectasis The presence (or absence) of confounding diseases needs to be dened, such as chronic obstruc4ve lung disease, inters44al lung disease, or malignancy.
Spirometry
Func4onal
assessment
of
pulmonary
impairment
Finding
Minimal
reduce
or
normal
FVC,
low
FEV1
are
most
nding
Very
low
FVC
can
seen
in
advance
disease
management
1. 2. 3. 4. 5.
Airway
hygiene
and
hyperosmo4c
agents
An4microial
therapy
An4-inammatory
therapy
Surgery
Cause
treatment
Mucus-mobilizing
method
Inhaled
beta-agonist/an4cholinergic
bronchodilator
Hypertonic
saline
or
manitol
inhala4on
Dornase
alfa
:
hydrolyze
neutrophil
DNA
benet
in
CF
and
may
be
benet
in
other
condi4on.
N-acetyl
cysteine
an4bio4c
Empirical oral ATB: Amoxycillin, Co-amoxiclav, Ciproox, : adjust depend on individual ,sputum Aerosol an4bio4c prevent exacerba4on: Tobramycin in CF (with Pseudo aeruginosa) pa4ent ,non CF are no ecacy
An4-inammatory
therapy
Goal
is
limit
progression
of
decline
lung
func4on
NSAID
Prostaglandin
play
role
in
augmen4ng
airway
secre4on
Inhaled
indomethacin
signicant
decreased
the
amount
of
sputum
(
prospec4ve
double
blind
) Cochrane
review.:High
dose
ibuprofen
slow
progressive
of
CF
especially
in
children
Inhaled
cor4costeroid
Tsang
ect.
Inhale
Flu4casone
1
yr:
decrease
sputum
,
no
eect
to
other
factor
Review
clinical
trial
in
CF
did
not
cause
improvement
Surgery
uncontrolled
hemoptysis
Remove
FB
or
obstruct
tumor
Focal
bronchiectasis
:
frequent
exacerbate
Lung
transplant
Indicate
in
end
stage
lung
Bilat
lung
transplant
or
heart
lung
transplant
Reference
1. Anne
E.
O Donnell;
Bronchiectasis;
Chest
2008;134;815-823
2. Alan
F.
Barker,M.D.
;
Bronchiectasis:
NEJM
2002
Vol.346,NO.18
;1383-93
3. Michael
D.
Iseman,
MD.
:Bronchiectasis
2010
Murray&Nadel s
Text
book
of
Respiratory
medicine
42:
1023-46
4. ;
Bronchiectasis 2550
:456-63