Fibrosis, Kartegener’s Syndrome or a1-antitrypsin deficiency.

So
PULMONOLOGY, MED II - 3RD YEAR these are the MAJOR ETIOLOGIES of your Bronchiectasis.
BRONCHIECTASIS
(DR CONSTANTINO TRANS MIDTERMS MARCH 2017) OTHER ETIOLOGIES:

Autoimmune or Rheumatoid arthritis, Sjogren’s

Rheumatologic syndrome, Inflammatory bowel
BRONCHIECTASIS Disease; immune-mediated
Disease (e.g., allergic broncho-
INFECTIOUS FOCAL Pulmonary aspergillosis)

Recurrent aspiration
Irreversible airway dilatation
Miscellaneous Yellow nail syndrome;
Traction bronchiectasis
NONINFECTIOUS DIFFUSE from postradiation fibrosis or
Idiopathic pulmonary fibrosis

CAYRAPF – MED II, PULMONOLOGY - MARCH 2017, 2ND SEMESTER

Bronchiectasis basically consists of irreversible airway dilatation Idiopathic
and it can be due to infectious or noninfectious causes, it can be
focal or diffuse. You can also have an autoimmune or rheumatologic conditions
like Rheumatoid arthritis, Sjogren’s syndrome, IBD, and immune-
FOCAL BRONCHIECTASIS DIFFUSE BRONCHIECTASIS mediated disease. Recurrent aspiration can also lead to recurrent
infections that can also develop to Bronchiectasis. Miscellaneous
conditions like Yellow nail syndrome, Traction bronchiectasis from
Localized Widespread changes
Throughout the lung post radiation fibrosis or idiopathic pulmonary fibrosis.

AREAS OF INVOLVEMENT
Consequences of obstruction Often arises from an underlying
of the airway systemic or infectious disease Upper lung fields Cystic Fibrosis (CF);
process Postradiation fibrosis

EXTRINSIC INTRINSIC Lower lung fields Chronic recurrent aspiration;;

End-stage fibrotic lung disease
Recurrent immunodeficiency
Compression from Airway tumor or associated infections
Lymphadenopathy or aspirated FB,
Parenchymal tumor a scarred/ stenotic airway, or Middle fields Infection by nontuberculous
mass bronchial atresia from congenital under mycobacteria;
development of the airway Dyskinetic/ Immotile Cilia
Syndrome
For Focal Bronchiectasis, this is localized and it is a consequence
Central airways Allergic bronchopulmonary
of airways obstruction. Obstruction can be intrinsic or extrinsic. If it
Aspergillosis (ABPA);
is extrinsic, you can have compression from lymphadenopathy or Tracheobronchomegaly
parenchymal tumor masses outside the bronchus. If it is intrinsic, (Mounier-Kuhn syndrome) &
you can have an endobronchial tumor or an aspirated foreign William Campbell Syndrome
body, a scarring or stenotic airway as in TB or bronchial atresia
from congenital underdevelopment of the airway. ETIOLOGY
With regards of the etiology of Bronchiectasis, for infectious
For Diffuse Bronchiectasis, you have widespread changes causes you can have viruses (Adenovirus & Influenza virus). For
throughout the lung. It usually arises from an underlying systemic concomitant bacteria, usually the necrotizing type of bacteria
or infectious disease process. such as Staph aureus, Klebsiella anaerobes and M. tuberculosis.
MAJOR ETIOLOGIES OF BRONCHIECTASIS A. INFECTIOUS CAUSE
a. Viruses – Adenovirus and Infleunza virus
DIFFUSE b. Bacteria – Staph. aureus, Klebsiella, Anaerobes, M.
tuberculosis
INFECTION Bacterial, Nontuberculous
Mycobacterial
Mycoplasma and necrotizing fungal infection – rare causes
IMMUNODEFICIENCY Hyper-gammaglobulinemia,
HIV infection, Bronchiolitis PATHOGENESIS & PATHOLOGY
obliterans, alter The most widely cited mechanism of infectious bronchiectasis:
lung transplantation “vicious cycle hypothesis,”
- In which susceptibility to infection and poor mucociliary
GENETIC Cystic fibrosis, Kartagener’s clearance result in microbial colonization of the
syndrome, α1- antitrypsin
bronchial tree – so you have developed a chronic
deficiency
inflammation.
For diffuse bronchiectasis the most common cause would be
bacterial infections or nontuberculous mycobacterial infections.
You can have immunodeficiency syndromes like Hyper-
gammaglobulinemia, HIV, Bronchiolitis obliterans, or post
transplant immunodeficiency. And genetic conditions like Cystic

Page 1 of 5
NORMAL HOST RESPONSE VICIOUS CYCLE

Insult INFLAMMATION
Infection
Underlying
condition Cytokines
Bacterial
Neutrophil enzymes colonization/
Bacterial products Infection

BRONCHIAL
DRAINAGE

HEALTH

MUCOCILIARY Mucociliary
CLEARANCE clearance
further impaired
NORMAL IMPAIRED

CAYRAPF – MED II, PULMONOLOGY - MARCH 2017, 2ND SEMESTER

MUCUS HYPERSECRETION
OBSTRUCTION

In the NORMAL HOST RESPONSE, you have normal mucociliary

clearance whenever you have an insult such as infection or other
underlying conditions. You are able to overcome this so eventually you DIFFERENT PATTERNS OF BRONCHIECTASIS (Described by Reid)
have a healthy airway or lung. But for the VICIOUS CYCLE, you have NORMAL (1) VARICOSE (4)
an impaired mucociliary clearance. You have mucus hypersecretion
leading to obstruction of the airway. You have further impairment of
mucociliary clearance due to excessive secretions and eventually you
have bacterial colonization and infection and the presence of
chronic inflammation with resultant production of cytokines, neutrophil
enzymes and bacterial products which are injurious to the lung tissue.
Eventually you have impaired drainage and you develop
Bronchiectasis.

Defect in the clearance mechanism

Accumulation of secretion

Bacterial overgrowth

Infection and atelectasis (Chronic Infection)

Destructive inflammation of bronchial wall

SACCULAR (2) CYLINDRICAL (3)
- So eventual damage to the bronchial wall leading to You have several types of Bronchiectasis. You have:
dilatations. That’s why it is called Bronchiectasis. Cylindrical (Tubular) Bronchiectasis wherein you have uniformly
dilated bronchial tubes.
PROPOSED MECHANISMS FOR NONINFECTIOUS BRONCHIECTASIS
Varicose Bronchiectasis, you have irregular or beaded pattern
For NON-infectious Bronchiectasis, you have proposed mechanisms:
so you have dilatation, constriction, dilatation, constriction.
IMMUNE MEDIATED Reactions that damage the bronchial wall Saccular (Cystic) Bronchiectasis, the bronchial tube ending in
(eg., those assoc. with systemic autoimmune line sacs parang cystic dilatation. So if you look at the
conditions such as Sjorgen syndrome & RA) bronchogram on your right so you have on top the cylindrical
bronchiectasis, on the lower right you have the varicose
TRACTION Refers to dilated airways arising from
bronchiectasis and on the lower left the Cystic Bronchiectasis
BRONCHIECTASIS parenchymal distortion as a result of lung
Fibrosis (e.g., postradiation fibrosis or IPF) parang grape-like yung clusters.

PATHOLOGY

Chronic, necrotizing infection of bronchi

Damages and weakens walls of bronchi Markedly dilated &

Filled with mucous
& pus

Dilated airways occluded by secretions or

Obliterated and replaced by fibrous tissue

Page 2 of 5

On cut sections, you have the dilated bronchial segments.
HISTOPATHOLOGIC CHANGES:
1. Bronchial and peribronchial inflammation and fibrosis
2. Ulceration of the bronchial wall
3. Squamous metaplasia
4. Mucus gland hyperplasia
5. Parenchyma supplied by the affected airways – contains
combination of fibrosis, emphysema, bronchopneumonia,
and atelectasis
6. Increased vascularity of the bronchial walls due to
inflammation with associated enlargement of bronchial
arteries and anastomoses between the bronchial and
pulmonary arterial circulation – This is very significant
because this may result in massive bleeding so hemoptysis
usually occurs in about 70% of cases of Bronchiectasis. It is
one of the problems in Bronchiectasis. The bleeding can be
massive enough to cause asyphyxia in the patient.
PRIMARY CILIARY DYSKINESIA (PCD)
 Associated with recurrent upper and lower respiratory tract
infections (because you have impaired ciliary movements,
you cannot clear your secretions, you have recurrent
infections) as sinusitis, otitis media, and bronchiectasis;
sperm motility affected leading to infertility among males –
So if you have a patient with Bronchiectasis, you ask about
the presence or absence of children. Because the
Bronchiectasis may be secondary to primary ciliary
dyskinesia.
 Half of patients with PCD fall into subgroup of Kartagener’s
Syndrome – which consists of a triad of situs inversus with
bronchiectasis and sinusitis – yan yung lagi niyong
kinakalimutan!
CYSTIC FIBROSIS (CF)
 A monogenic (recessive genetic) disorder
 Presents as a multisystem disorder characterized by chronic
airway infection (This is due to tenacious secretions) leading
to
o Bronchiectasis and Bronchoatelectasis
o Exocrine pancreatic insufficiency & intestinal
dysfunction
o Abnormal sweat gland function, and
o Urogenital dysfunction
 Cause transmutation in the gene for protein CFTR (required
for regulation of sweats, digestive juice, mucus)
CLINICAL MANIFESTATION OF BRONCHIECTASIS
Of course, the most obvious is recurrent cough and purulent
sputum production. Usually you have copious amount of sputum
expectorated on waking up in the morning kasi nagppool iyan
while they are sleeping. Umiipon yung sputum so pagkagising
they have to cough it out.
 Hemoptysis – 70% of cases
Page 3 of 5
 Dyspnea or wheezing – Because Bronchiectasis is more of
an obstructive lung disease. You may hear on PE a very
noisy chest with crackles, ronchi and wheezes. Sometimes
this is localized if you have Localized Bronchiectasis.
 Clubbing of fingers – chronicity of the lungs
 Severe, diffuse disorder, chronic hypoxemia, may have
associated cor pulmonale and signs of RV failure like
bipedal edema
 Crackles, ronchi, wheezes
 Recurrent cough and purulent sputum production
DIAGNOSIS
1. Chest radiography
2. CT scan - With the advent of High resolution CT scan in
multiple slices we are able to visualize even the linear or
tubular type of Bronchiectasis. So the CT scan is used
CAYRAPF – MED II, PULMONOLOGY - MARCH 2017, 2ND SEMESTER
for diagnosis of Bronchiectasis. – Dx of choice
3. Bronchographyv – Before we used to do
Bronchography wherein you still have to incorporate
dye in the bronchus and then take several X-rays but
this is a very tedious and awkward procedure.
4. Bronchoscopy – not required for the diagnosis of
Bronchiectasis because it only shows you an airways
with a very thin mucosa. This is done if you are trying to
rule out the possibility of obstruction.
CHEST RADIOGRAPHY
 Often nonspecific; normal with mild disease – so even if you
hear crackles, in the CXR it can be normal especially if it is a
linear or tubular type of Bronchiectasis
 Saccular bronchiectasis – with prominent cystic spaces,
either with or without air-liquid levels, corresponding to the
dilated airways
 Dilated airways seen as
o “tram tracks” longitudinally – parang railways
o “ring shadows” x-sectionally due to decreased
aeration and atelectasis fo the associated
parenchyma – so parang may ring density. On top
of the ring, you have a vessel so parang signet-
ring.
 Dilated airways filled with secretions appear dense
producing an opaque tubular or branched structure
RING SHADOWS
(Pictures on the next page) Nakikita niyo ba ang cystic
dilatations? Sa upper left, left lower lung field you have a cystic
dilatation. On your lower right, it is in the right lower lung field. If
you look behind the cardiac shadow on the left, makikita mo
parin na may mga cystic lucencies. You always have to look at
behind the cardiac shadow, ganun din siya sa peripheral lung
field sa left.
 COMPUTED TOMOGRAPHY (CT SCAN)
 Provides excellent view of dilated airways as seen in cross-
sectional images
 Can suggest specific etiology of the bronchiectasis, (ex.
Bronchiectasis of relatively proximal airways suggests ABPA,
presence of multiple small pulmonary nodules (nodular
bronchiectasis) suggests infection with M. avium complex

CT FINDINGS
 Airway dilataion (detected as parallel “tram tracks” if they
are seen longitudinally or as the “signet-ring sign” – a cross-
sectional area of the airway with a diameter at least 1.5
times that of the adjacent vessel
 Lack of bronchial tapering (including the presence of
tubular structures within 1 cm from the pleural surface)
So if you have the bronchial wall thickening with secretions you

CAYRAPF – MED II, PULMONOLOGY - MARCH 2017, 2ND SEMESTER

This is a picture taken from a different source. I hope this will help: term that as the TREE-IN-BUD pattern,
NORMAL BRONCHIECTASIS

This is a comparison in the CT scan of your normal lung and

Bronchiectatic lung. So you can see the dilated bronchial segments
there.

APPROACH TO PATIENTS WITH BRONCHIECTASIS

1. Chest imaging – chest x-ray and/or chest CT scan
2. Bronchoscopy – BAL (Bronchoalveolar Lavage)
3. Gram’s stain / culture
4. Stains/ cultures for acid-fast bacilli and fungi
5. Complete blood count with differential
6. Immunoglobulin measurement
7. HIV testing
8. Measurement of chloride levels in sweat – for CF
9. Measurement of a1 antitrypsin levels – if you suspect a1
antitrypsin deficiency
10. Nasal or respiratory tract brush/biopsy (for dyskinetic/
immotile cilia syndrome)
11. Genetic testing
12. Serologci testing (eg., for rheumatoid factor)
13. Consider workup for ABPA, especially in patients with
refractory asthma
14. Test of swallowing function and general neuromuscular
BRONCHOGRAPHY strength – in rec. aspiration
 Visualization of bronchiectatic airways TREATMENT: BRONCHIECTASIS
 Coating the airways with radiopaque, iodinated lipid dye  Directed at the control of active infection
instilled through a catheter or bronchoscope  Improvements in secretion clearance and bronchial
hygiene to
o Decrease the microbial load within the airways
and
o Minimize the risk of repeated infections
So this is done by postural drainage.

ANTIBIOTIC TREATMENT
 Targeting the causative or presumptive pathogen (with H.
influenza and P. aeruginosa isolated commonly) should be
administered in acute exacerbations, usually for a minimum
of 7-10 days – you should cover for Pseudomonas always

BRONCIAL HYGIENE includes:

 Hydration and mucolytic administration

Page 4 of 5
 Aerosolization of bronchodilators and hyperosmolar agents
(eg., hypertonic saline)
 Chest physiotherapy
o Postural drainage
o Traditional mechanical chest percussion via hand
clapping to the chest, or
o Use of devices such as oscillatory positive
expiratory pressure flutter valve or a high-
frequency chest wall oscillation vest
 The mucolytic domase (DNase) is recommened routinely in
CF-related bronchiectasis but not not in non-CF
bronchiectasis
ANTI-INFLAMMATORY THERAPY
 Use may
o Alleviate dyspnea – so it means steroids
o Decrease need for inhaled beta-agonists, and
o Reduce sputum production with inhaled
glucocorticoids
 Oral/systemic glucocorticoids may be important in
treatment of ABPA (Allergic Bronchopulmonary
Aspergillosis), or noninfectious bronchiectasis associated
with RA (Rheumatoid Arthritis) or Sjorgen’s syndrome
 Patients with ABPA may also benefit from a prolonged
course of treatment with the oral antifungal agent
Itraconazole
Massive hemoptysis
This is defined as coughing out of blood at least 250 ml or more
than 600 ml in 24 hours.
 If not resolve with rest and antibiotics, therapeutic options
are either:
o Surgical resection (for localized lesion)
o Bronchial arterial embolization (for widespread
disesase)
Chronic hypoxemia/ Cor Pulmonale
 Long term O2 therapy
Lung Transplant
 For selected patients who are disabled despite maximal
therapy
COMPLICATIONS OF BRONCHIECTASIS
Because of the decreased ability to mobilize secretions.
 Recurrent infections
 Decreased sputum production
 Leading to Dyspnea
 Decrease air emptying with decreased oxidation of tissues
 A decrease in oxygen levels over time leading to
constriction of pulmonary arteries
 Decreased pressure in pulmonary arteries leading to
Pulmonary Hypertension
 Pulmonary Hypertension leads to overload in the heart, you
have RVH, Cor pulmonale
 Bipedal edema, ascites and neck vein distention
So the patient becomes hospitalized because of these
complications.
PROGNOSIS
 Bronchiectasis is not curable – It is a permanent anatomic
defect
 Early recognition and adequate treatment can help control
bronchiectasis and decrease symptoms – because it is
usually progressive with recurrent infection you develop
more areas of Bronchiectasis. As much as possible you try to
control infection to prevent worsening of the Bronchiectasis.
Page 5 of 5
 Life-long awareness of the need for treatment may allow
people with bronchiectasis to minimize complications and
maximize life expectancy
 Congenital causes of bronchiectasis, like CF, may have a
worse prognosis than acquired diseases – So usually in these
patients with congenital conditions will not live that long
PREVENTION
 Reversal of an underlying immunodeficient state (e.g., by
administration of gamma globulin for immunoglobulin-
deficient patients)
 Vaccination of patients with chronic respiratory conditions
(e.g., influenza and pneumococcal vaccines)
o Can decrease the risk of recurrent infections
 Smoking cessation
CAYRAPF – MED II, PULMONOLOGY - MARCH 2017, 2ND SEMESTER
POSSIBLE SUPPRESSIVE TREATMENTS INCLUDE:
(1) Administration of an oral antibiotic (e.g., ciprofloxacin)
daily for 1-2 weeks per month, referred as cyclic
antibiotics but usually this is reserved for patients with
cor pulmonale
(2) Use of a rotating schedule of oral antibiotics (to
minimize the risk of development of drug resistance),
(3) Administration of a macrolide antibiotic daily or three
times per week (with mechanisms of possible benefit
related to non-antimicrobial properties, such as anti-
inflammatory effects and reduction of gram-negative
bacillary biofilms),
(4) Inhalation of aerosolized antibiotics – but this is very
expensive
e.g., tobramycin inhalation solution (TOBI)] by select
patients on a rotating schedule (e.g., 30 days on, 30
days off) with the goal of decreasing the microbial
load without encountering the side effects of systemic
drug administration
(5) Intermittent administration of IV antibiotics
(e.g., “clean-outs”) for patients with more severe
bronchiectasis and/or resistant pathogens
SUMMARY
 Definition of Bronchiectasis
 Pathological Findings – Gross and Microscopic
 Different forms
 Etiology
 Clinical Manifestations
 Laboratory Examinations
 Treatment/Prevention
WHAT ARE THE 3 FORMS OF BRONCHIECTASIS?
(1) SACCULAR/ CYSTIC – Anong saccular at cystic!
Magkapareho lang yan! Saccular or cystic!
(2) VARICOSE
(3) CYLINDRICAL/ TUBULAR
*May natatandaan pala kayo!*
-LIGHT-
Please do not rely on this. Double check for any mistakes. Read
the book and study well. God bless, doctors! Thank you.
CAyraPF