Dr.

Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

BRONCHIECTASIS
DEFINITION
Bronchi ectasis i s a congeni tal or acqui red di sorder of t he
l ar ge bronchi charact er ized by per manent , abnormal
dil ation and dest ruct ion of bronchial wal ls. It may be
caused by recurrent i nf lammat ion or i nfecti on of the
air ways and may be l ocali zed or dif fuse.
ETIOLOGY
L UNG INF ECTION
T uber culosis, fungal infections, lung abscess, pneum onia
GENERAL IZED IMPAIRM ENT OF PUL MONARY DEFE NS E
M ECHANISMS
Immunoglobulin deficiency, primar y ciliary disorders, or cystic
fibrosis
L OCALIZED IMP AIRMENT OF HOS T DE FENSES
F or eign body, tumor, mucoid im paction
E XCE SSI VE IMMUN E R ESP ONS E
All ergi c bronchopul monary aspergil losis
T OXI C IN SUL T
DE VE LOP MENT AL DEF EC TS
Williams–Cam pbell syndrome
M ounier –Kuhn syndr om e or tr acheobr onchomegaly
Pulmonar y sequestr ation
AS SOC IAT ED CONDI TI ONS
R heumat oi d ar thrit is
I nf lammat or y bowel di sease
I DIOP ATH IC BRONC HIE CT ASIS

PAT HOLOGY
MACR OSC OPIC IN SPE CTI ON:
• P er manent di latati on of subsegment al air ways that are
i nf lamed and of ten part iall y or totall y obstructed wi th
secret ions.
Dr. Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

• P AT TER NS
– Cylindr ical br onchiectasis
– Var icose br onchiectasis
– Saccular (cystic) bronchiectasis

MIC ROSC OPIC F EAT URE S:

• Chronic i nf lammat ion in t he bronchial wall wi th
i nf lammat ory cel ls and mucus in the l umen.
• T here i s destructi on of the el ast in l ayer of t he bronchi al
wal l with a variable amount of fi brosi s.
• Neutrophi ls are t he main cell populati on i n the bronchi al
l umen, whereas the commonest cell s in t he bronchial wall
are mononucl ear.

P ATHOGENESIS
…………..Discussed

CLINICAL FEATURES
S YMPT OMS
Chronic cough wi th production of copious amounts of
purul ent sputum (postural variati on), Hali tosis
Hemopt ysi s (50–70% )
D yspnea and wheezi ng ( 75%)
P leur iti c chest pain
F at igue, weight l oss, and myal gi as

SI GNS
• Cl ubbing
• S igns of causat ive condit ions
• Cr ackles
• Wheeze
Dr. Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

• Cor pul monal e and ri ght ventri cul ar f ai lure. (Wi th severe
dif fuse di sease)
INV ESTIGATIONS
ROUTINE
• Ch est radiog raph
May be norm al
Tr am tracks
Ring shadows
Br anched tubular str ucture
Cystic spaces (saccular bronchiectasis)
• High -r esolut io n CT scan (lun g an d sinu ses)
• Complete blood count
• E SR, C-r eactive protein
• Gram st ai ni ng and cul turing of sputum
• Mycobacterial sputum cul ture
• F ungal sput um cul ture
• P ul monary funct ion tests

SPECIFIC
• F iber optic bronchoscopy
• Sweat chlor ide
• CF genotyping
• Im munoglobulin ( Ig) levels
• Aspergillus skin testing
• Ciliary morphology or function
• Sem en analysis
• Bar ium swallow
• pH pr obe
• Alpha1-antitr ypsin levels
• Rheumatic disease ser ology

TREATMENT
Goal s:
Tr eatm ent of infection, par ticularly duri ng acute exacerbations
Dr. Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

Impr oved clear ance of tr acheobr onchial secr etions

Reduction of inflamm ation
Tr eatm ent of an identifiable under lying probl em

• Antibiotics
• Physiother apy
• Sur gical ther apy
• Br onchial arterial embolization (for non resolving massive
hemoptysis )

PREVENTION
• Adequate pr ophylaxis and treatment of m easles, whooping cough
or tuberculous infection
• Ear ly r ecognition and treatm ent of br onchial obstruction

P ROGNOSIS
• Ci l iary dysfunct ion and cystic fibrosis: Progressive disease
eventuall y causes r espiratory f ai lure
• Ot her pati ents t he prognosis can be r el at ively good wi th
post ur al drai nage and antibioti cs

CY STIC FIBROSIS
GE NE RAL
• A ut osomal recessi ve di sorder
• Caused by abnormal it ies i n a membrane chloride channel
(t he cysti c fi brosis t ransmembrane conductance r egulator
[ CFT R] prot ei n) that results i n altered chl or ide transport
and wat er fl ux across the api cal surf ace of epitheli al cell s.
• Mutati on referred t o as ▲F 508 accounts f or about 60% of
cases of cysti c fi br osis.
Dr. Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

P ATHOGENESIS
• A lmost all exocri ne glands produce an abnor mal mucus
t hat obstructs glands and ducts.
• Obstructi on resul ts i n glandular dil ation and damage to
t issue.
• I n the respiratory tract , i nadequat e hydrati on of t he
t racheobronchial epi thel ium i mpair s mucocili ary funct ion.
CLINICAL F EATURE S
• Chronic or recurrent cough, sput um producti on, dyspnea,
and wheezing.
• Recurrent i nfecti ons or chroni c col oni zati on of the ai rways
wi th nont ypeable H i nf luenzae, mucoid and nonmucoid
P seudomonas aeruginosa, St aphylococcus aureus, or
B urkholder ia cepacia.
INV ESTIGATIONS
• Chest radiographs: B ronchi ect asi s
• S pi romet ry: A irf low obst ruct ion
• S weat chl oride concentrati on above 60 mEq/L on two
occasi ons
• Gene mut at ion known to cause cystic fibrosis.
TREATMENT
GOAL S:
• Clear ance and reduction of lower airway secretions
• Rever sal of bronchoconstr iction
• T reatm ent of r espir ator y tr act infections and air way bacter ial
burden
• Pancreatic enzym e r eplacement, and nutr itional and psychosocial
support (including genetic and occupational counseling) .

• Lung tr ansplantation is curr ently the only definitive tr eatment for

advanced cystic fibrosis.
• Investigational ther apies:
Dr. Ra h ull Mag ag a zi n e
M .D..((Meed iccinne ); D.T.C.D.
De ptttt.. o f Resp
sp irat
ato ry Me
Me d ic in e

-Anti-inflamm atory agents (eg, ibuprofen, pentoxifylline,

antipr oteases)
-Pr otein modificati on agents ( eg, milr inone, phenylbutyr ate)
-Ion transpor t agents ( eg, amiloride)
-Gene ther apy.

P RO GNOSIS

• T he l ongevi ty of pati ents w ith cysti c fi brosis i s i ncreasi ng,

and the median sur vi val age is over 30 years.
• Death occurs f rom pul monar y compli cat ions (eg,
pneumoni a, pneumot horax, or hemoptysis) or as a result
of terminal chroni c respi rat or y fail ure and cor pul monale.