BRONCHIECTASIS

- IRREVERSIBLE airway dilation that involves the lung either: FOCAL OR DIFFUSE
- A Chronic disease
- 25-50% idiopathic in origin
- Loss of airway tone results in air trapping
- Na compare siya between straw and a big pipe. If I ask you to blow a straw and nilagay ko yung
daliri ko sa kabilang side I can feel the air. Pero pag hinarap kita sa big pipe and sabi koi blow mo
I wont be able to feel the air kasi yung air paikot ikot nalang sa loob nang tude so hirap kang
ilabas ( air will acelarated and roll against the wall of the dilated bronchi.

Focal bronchiectasis: bronchiectatic changes in a localized area of the lung and can be consequence of
obstruction.

o Extrinsic focal bronchiectasis – due to compression by adjacent lymphadenopathy or

parenchymal tumor moss
o Intrinsic focal bronchiectasis – due to an airway tumor or aspirated foreign body, a
saccered/ stenotic airway or bronchial atresia from congenital underdevelopment of the
airway

Diffuse bronchiectasis: wide spread bronchiectasis changes throughout the lung often arises from an
underlying systemic or inf disease

Categories:

1. Cylindrical or tubular bronchiectasis - most common, the bronchi appear as uniformly dilated
tubes that end abruptly at the point that smaller airways are obstructed by secretion
2. Varicose bronchiectasis – the affected bronchi have irregular or beaded pattern of dilation
resembling varicose veins.
3. Saccular or cystic bronchiectasis - the bronchi have a ballooned appearance at the periphery,
ending in blind sac without recognizable bronchia structure distal to the sacrs

Etiology and there common location:

1. Upper lung field involvement: common in cystic fibrosis, observed in post radiation fibrosis
corresponding to the lung region encompassed by radiation port
2. Lower lung field involvement: source chronic recurrent aspiration ( esophageal motility)
- End stage fibrotic lung disease
- Recurrent immunodeficiency associated infection ( hypogammaglobulinemia
3. Mid lung field involvement: infection by non- tuberculosis mycobacteria ( MAC) –
mycobacterium avium complex.
4. Congenital + midlung involvement: dyskinetic, immotile cilia syndrome.
5. Central airway involevement: assoc. with allergic bronchopulmonary aspergilosis in which
immune mediated reaction to aspergillus damage the bronchial wall
6. Congenital + central airway: cartilage efficiency include tracheobronchomegaly
- Mounier- kuhn syndrome and Williams-campbell syndrome)
Etiology and proposed workup

1. Focal  di ba sabi ko kanina pag focal isang area lang pero its either intrinsic which is yunf mga
airway tumor, aspirated foreign body or stenosis or pwede din extrinsic na dahil nacompress
dahil sa lymphadenopathy. So inshort more OBSTRUCTION ANG ETIOLOGY  you do:
a. Bronchoscopy
b. Chest imaging ( xray or ct scan)
2. Diffuse
a. Infection in origin  sputum g/s , c/s and AFB if no pathogen seen  bronchoscopy with
bronchoalveolar lavage
b. Immunodeficiency ( hypogamma,HIV, bronchiolitis)  CBC with diff count, immune
globulin measurement and hiv testing
c. Genetic cause (CF, kartageners)  chloride test for cf, respitract biopsy, alpaha 1 trypsin
testing and genetic testing
d. Autoimmune or rheumatologic cause, immune mediated and recurrent aspergillosis
joint examination, serologic testing, aspergillosis work up and swallowing finction for
recurrent aspiration

PATHOLOGY:

- In bronchiectasis the affected airway is the BRONCHI which is a relatively large airways with
affected supporting structures.
- Bronchiectasis is sometimes preceded by pneumonia which was not treated early

Gross
- Airway affected are the medium sized
airways, often at the level of the
segmental and subsegmental
- Cartilage and muscles and elastic tissues
are destroyed and replaced by fibrous
tissue
- Pools of thick purulent material in the
dilated airways
- Peripheral airways are obstructed by
secretion or obliterated and replaced by
fibrous tissue
microscopic
- bronchial and peri bronchial
inflammation and fibrosis
- ulceration of the bronchial wall
- squamous metaplasia
- mucus gland hyperplasia
- fibrosis, emphysema, bronchopneumonia
and atelectasis of the lung parenchyma
- inc vascularity of the bronchial wall with
enlargement and anastomoses between
the bronchial and pulmo arterial
circulation

PATHOGENESIS:

- VICOUS CYCLE HYPOTHESIS – most widely cited mechanism of infectious bronchiectasis, in

which susceptibility to infection and poor mucociliary clearance result in microbial colonization
of the bronchial tree
- Bronchiectasis is a consequence of inflammation and destruction of the structural component of
the bronchial wall. INFECTION IS USUALLY THE CAUSE OF THE INFLAMMATION
MICROORGANISM  RELEASES PIGMENTS, PROTEASES, TOXINS  RESPIRATORY EPITHELIUM INJURY
AND IMPAIRED MUCOCILLIARY CLEARANCE  INFLAMMATORY REACTION: MEDIATORS RELEASE
FROM NEUTROPHILS ( INFLAMMATORY CELLES RELEASES ROS AND OTHER CYTOKINES  ADDS
DAMAGE OR INJURY TO THE LARGER AIRWAYS AND OBSTRUCTION IN SMALLER AIRWAYS)
EPITHELIAL INJURY COMPROMISED DEFENSE MORE SUSCEPTIBLE FOR COLONIZATION AD
GROWTH OF BACTERIA

- Some organism such as PSEUDOMONAS AERUGINOSA—exhibit a particular propensity fo

colonizing damaged airways and evading host defense mechanism.
- Impaired mucociliary clearance can result from inherited condition such as CF or dyskinetic cilia
syndrome and it has been proposed that a single severe infection can result in significant airway
damage and poor secretion clearance
- Presence of the microbes incites continued chronic inflammation, with consequent damage to
the airway wall, continued impairment of secretion and microbial clearance and ongoing
propagation of the inflame cycle.

NORMAL DEFENSE MECHANISM OF THE BODIES THAT TURNS OUT TO BE INDUCERS 

1. Neutrophils -- primary inflammatory cells in response to infection to the airway

- Secretes inflammatory mediators
a. Proteases
b. Metalloproteases
c. ROS and other cytokines
(that all contribute to the damage in the larger airways)

2. Mucociliary clearance mechanism- it is the most important defense mechanism of the airways,
so if this is impaired microbes will undergo STASIS more susceptible for bacteria.
 3. Pool of thick secretion in the airways- good medium for the microorganism to grow
4. Anti- proteases such as alpha 1 antitrypsin – play an important role in neutralizing the
damaging effects of neutrophil elastase and in enhancing bacterial killing. Which bronchiectasis
and emphysema have been observed antitrypsin deficiency
5. Immune mediated reaction – damage the bronchial wall assoc with systemic autoimmune
condition ( sjogren syndrome and rheumatoid arthritis)

Traction bronchiectasis – refers to dilated airways arising from PARENCHYMAL DISTRUCTION as a result
of lung fibrosis.

Infection causes:

a. Adenoviruses and influenza virus  MOST e. Mycobacterium tuberculosis – impt cause

COMMON of bronchiectasis ( upper airways)
b. Staphylococcus aureus f. MAC
c. Klebsiella g. Mycoplasma – fungal most commonly
d. Anaerobic bacteria histoplasma
( B-D  causes necrosis)

IMPAIRED HOST DEFENSE MECHANISM  prone to develop Bronchiectasis

1. Endobronchial obstruction (major cause)

o Panhypogammaglobulinemia
- presents w/ a lot of skin lesion on the head or body
o IgG subclass immunoglobulin deficiency

Endobronchial obstruction (localized type)

- may be due to a tumor in the airway or aspirated foreign body; tumor may be outside the
airway causing external compression of the airway leading to obstruction – ex. Px w/ primary
complex w/ enlarged lymph notes & infiltrates in the lung parenchyma
- located at the lower lobe instead of the upper lobe

- Patients w/ large lymph nodes – usually located at the mediastinal or hilar area leading to
airway obstruction

2. Primary ciliary disorder / Primary ciliary dyskinesia (generalized type of Brondhiectasis)

- 5 – 10% of cases of bronchiectasis
- Kartagener’s Syndrome – autosomal recessive hereditary disease
- sinusitis
- bronchiectasis
- situs invesus
- during the developmental stage of fetus, most of the internal organs are located on the left side
- fetus need the ciliary action to be able to twist around to its normal location
- if there is a problem w/ the cilia, the internal organs will not be able to twist around
- may be incomplete where in some patients, the heart may be in abnormal position
- Most patients’ (male) sperm cannot swim (infertile)
3. Cystic fibrosis mucoid strain
 P. aeroginosa
 S. aureus
 H. influenza
 E. coli
 Burkholderia cepacia

- patients have very tenacious secretions (cannot cough out)

- before, patients usually die during childhood

Non-infectious Causes: (generalized type of Bronchiectasis)

- Toxic gases – ammonia

- Acidic gastric content (aspiration)
- Immune response
o Aspergillosis (ABPA – Allergic Bronchopulmonary Aspergillosis)  can cause destruction
of the airway
o Ulcerative colitis
o Rheumatoid arthritis
o Sjogren’s syndrome
- Alpha-1 antitrypsin deficiency increase the risk of developing emphysema
- Yellow nail syndrome  problema with the lymphatic drainage
o Lymphedema
o Pleural effusion
o Yellow discoloration of the nail 40% of patient have bronchiectasis
CLINICAL MANIFESTATION:
1. COUGH- persistent productive cough with ongoing production of thick, tenacious sputum
- due to irritation and inflammation of the airway
- COPIOUS AMOUNT OF SPUTUM ( GLASSFUL AMOUNT PER DAY)
2. CRACKLES , WHEEZING AND RHONCHI
Crackles – opening of the alveoli; fluid w/ in the alveolar level  respi bronchiole and alveolar
sac
Wheezing – caused by any airway obstruction or narrowing
Rhonchi – caused by movement of secretion in larger airways
3. CLUBBING OF THE FINGERS – usually found Inpatients w/ congenital heart failure --. Presence
of hypoxemia
- Caused by chronic ingflammation
4. HEMOPTYSIS -- (50-70%) secondary to friable inflamed mucosa, hypertrophied bronchial
arterty
a. Minimal – secondary to the friable inflamed mucosa during infection
- The more inflame changes, the more the mucosa becomes friable
b. Massive – if bleeding comes from hypertrophied bronchial arteries
5. DYSPNEA – due to fibrosis causing obstruction and secretion contributing to airway obstruction
6. COR PULMONALE – due to increase pressure
- Disease of the heart secondary to an underlying lung problem
*** mild to moderate airflow obstruction is often detected on pulmonary function test, overlapping
with that seen at presentation with other condition such as COPD

*** acute exacerbation of bronchiectasis are usually characterized by changes in the nature of sputum
production, with inc volume and purulent

*** dry type of bronchiectasis : no building up of secretions since there is normal drainage in the upper
lobe assuming an upright position

DIAGNOSTICS:

we suspect patients to have bronchiectasis is they present with persistent-productive cough

-no consult by this time, will only seek consult after a year of coughing or when worst : hemoptysis.

1. Chest Radiograph
2. Bronchography
3. CT Scan
4. Bronchoscopy

1. Chest Radiograph

Early stage of bronchiectasis – normal CXR finding at early stage of bronchiectasis

- But positive rales, crackles, wheezes and rhonchi

 Often nonspecific; normal with mild disease

 Saccular bronchiectasis – with prominent cystic spaces, either with or without air-liquid levels,
corresponding to the dilated airways.
Severe bronchiectasis
 Dilated airways seen as :
o “tram tracks” longitudinally, vertical view
- Rail road track
- Airway filled up with secretions
- Casting a white shadow in CXR ( radio opaque)
- Airways running along side by side ( parallel to each other)
- Dilated airways filled with secretions appear dense producing an opaque tubular or
branched tubular structure
o “ring shadows” cross-sectionally due to decreased aeration & atelectasis of the
associated parenchyma , sagittal view
o Honey combing appearance
o Cystic sign
- Bunch of grapes seen in saccular types of bronchiectasis
- Dilation of cystic lesion
- Some are w/ airfluid level
o White lines
- Blood vessels that travels together with the airway
 2. Bronchography

- Visualization of bronchiectactic airways

- Definitive diagnostic before the advent of ct scan
- Coating the airways with radiopaque, iodinated lipid dye instilled through a catheter or
bronchoscope
- Disadvantage: allergic reaction to lipid dye → bronchospasm
- How: Instill dye → x-ray casting a shadow
- Use catheter into the lining and inject the dye ( contrast material
- Easily dries up – patient breathes the powder
3. CT Scan (gold standard)
- Provides excellent view of dilated airways as seen in cross-sectional images
- Can suggest specific etiology of bronchiectasis, (e.g. Bronchiectasis of relatively proximal airways
suggests…)

CT Findings:
 Airway dilatation (detected as parallel “tram tracks”)
 Bronchial wall thickening in dilated airways, inspissated reactions ( e.g. the “tree-in-bud”
pattern)
 Cysts emanating from the bronchial wall (especially pronounced in cystic bronchiectasis)

4. Sputum Exam
- May reveal abundant neutros & colonization of infection with variety of possible organisms
- Appropriate staining & culture often provide a guide to antibiotic therapy

5. Bronchoscopy
- Reveal underlying endobronchial obstruction in focal bronchiectasis
- Done if with hemoptysis, will administer agent to stop the bleeding

Other tests:
 Measurement of sweat chloride levels for cystic fibrosis
 Structural or functional assessment of nasal or bronchial cilia or sperm for PCD
 Quantitative assessment of immunoglobulins may explain
the recurrent airway infection
 Skin testing, serology & sputum culture for Aspergillus in
ABPA-related bronchiectasis
 PFT may demonstrate airflow obstruction as a consequence
of diffuse bronchiectasis or associated COPD
o PFT:
 FEV1:FVC ratio
 RV, FRC
 Normal FVC
 ↓ FEV1: ↓ FVC ratio
* ↓ FVC – not as low as restrictive pattern
*Obstructive type – bronchiectasis, COPD, asthma,
bronchiolitis
 Serologic evaluation – fungal infection and viral infection
  GERD test – causing aspiration upon backflow of acid

TREATMENT:
4 Major Goals:
1. Elimination of an identifiable underlying problem
2. Improve clearance of tracheobronchial secretions
3. Control of infection, particularly during acute exacerbations
4. Reversal of airflow obstruction

1. Elimination
 Treatment of hypogammaglobulinemia with Ig treatment

2. Improve clearance of tracheobronchial secretions

 Use of mucolytic agents to thin secretions & allow better clearance – N-acetylcysteine
(controversial)
 Aerosolized recombinant DNAse, which decreases viscosity by sputum by breaking down DNA
released from neutros – shown to improve pulmonary function in CF

3. Control of infection, particularly during acute exacerbations

 Antibioitcs important role in management
 Antibiotics are commonly used only during acute episodes
 Used in patients with chronic purulent sputum despite short courses of antibiotics,
4. Reversal of airflow obstruction
 Bronchodilators to improve obstruction & clearance of secretions particularly useful in patients…

*Massive hemoptysis
 Complete bedrest and antibiotics
 If it still persists, surgical resection and bronchial arterial embolization

*Chronic hypoxemia/Cor pulmonale

 Long term oxygen therapy

*Lung transplant
 For selected patients who are disabled despite maximal therapy

I. Antibiotic treatment:
- Antibiotic targeting causative or presumptive pathogen ( with H. influenza and P aeruginosa
commony isolated) should be administered in acute exacerbation usually for a minimum of 7-10
days and perhaps for as long as 14 days.
- Consensus guidelines have advised that diagnostic criteria for true clinical infection with NTM
should be considered
o in patient with symptoms and radiographic findings of lung disease
o who have at least 2 sputum samples positive in culture
o at least one bronchoalveolar lavage BAL fluid sample positive on culture
o biopsy sample displaying histopathologic features in NTM infection along with one
positive sputum culture or a pleural fluid sample positive on culture
 - MAC strain are the most common NTM pathogens and the recommended regimen for HIV
negative patients include  MACROLIDE COMBINED WITH RIFAMPIN AND ETHAMBUTOL
- IF P. AERUGINOS --. QUINOLONE OR AMINOGLYCOSIDES 3RD GEN CEPAHLOSPHORIN

II. Bronchial hygiene

- To enhance secretion clearance
a. Hydration
b. Muclytic administration
c. Aerosolation of bronchodilators
d. Hyperosmolar agents
e. Chest physiotherapy
f. Pulmonary rehabilitation – may assist with secretion clearance as well as with other aspects
including capacity and QOL
g. Mucolytic dornase ( DNase) recommended routinely in CF related bronchiectasis but not in
non CF given concerns about lack of efficacy and potential harm in the non CF population
III. Anti - inflammatory therapy
GLUCOCORTICOIDS
- This alleviate dyspnea, decrease need for inhaled beta agonist and reduced sputum production
*** risk of immune suppression and adrenal suppression must be carefully considered with use of
anti inflam therapy in infectious bronchiectasis
*** admin of oral/ systemic glucocorticoids may be important in treatment of bronchiectasis due to
certain etiologies such as ABPA

COMPLICATIONS
 Decrease ability to mobilize secretions → recurrent infection → increase sputum production
 Eventually, RV may fail

Hospitalization – Required in the ff cases:

1. Pneumonia
2. Massive hemoptysis
3. Respiratory failure
4. Heart failure

Prognosis:
 Bronchiectasis is not curable
 Early recognition & adequate treatment can help control bronchiectasis & ↓ symptoms
 Lifelong awareness of the need for treatment