IM-A: PULMONOLOGY

2.3 BRONCHIECTASIS
Dr. Pio Esguerra II, FPCP, FPCCP
Date: August 27, 2015
FEU-NRMF MEDICINE 2017
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BRONCHIECTASIS  Mycobacterial and non-mycobacterial infections is the
 Bronkia (bronchial tubes), ek (out) and tasis (stretching) MOST COMMON CAUSE in the Philippines
- “ outstretching of the bronchi”  So it is wrong to always associate HEMOPTYSIS
 Abnormal and permanent dilatation of bronchi, may be with TB, because the most common cause of
either focal, involving airways supplying limited region HEMOTYSIS is BRONCHIECTASIS (LALABAS TO!)
of the pulmonary parenchyma, or diffuse, involving  TB and non-mycobacterial infections may significantly
airways in a more widespread distribution increase the prevalence of bxsis bec these infections
 Localized like in Tuberculosis are known to cause bxsis , sometimes even when
 represents a chronically symptomatic disease. treated with antimicrobial agents.
CENTRAL ISSUE
 Infection as proximate cause OR
 Infections develop because of underlying predisposing
conditions
 central issue in understanding the pathogenesis of bxsis
is whether infections is truly the proximate cause of
bxsis or patient develops infectionas but bear in mind
that bxsis is caused not only by infectious but non-
infectious processes as well.
CLASSIFICATIONS AND PATTERNS

Imagine a lung that with a lot of mucus inside this is a

BRONCHIECTATIC LUNG. The lung is thickened, hypertrophied
and can be fibrotic. With increased mucus secretion giving the
main problem of too much coughing and phlegm.
The lungs are permanently dilated, scarred and thickened walls
present clinically as recurrent, chronic or refractory infections,
hemoptysis airflow obstruction and progressive impairment of
CYLINDRICAL SACCULAR VARICOID
breathing.
 Failure  Focal  Marked
EPIDEMIOLOGY
of the involved or cystic by irregular
 no systematic data on the incidence or prevalence
airways to taper distortion of dilation,
 because it is a manifestation of so many
progressively in distal airways narrowing, and
symptoms and disorders of the respiratory
their distal  May outpouching of
tract
course be isolated the airways
 Introduction of antibiotics and vaccines in 20th century
 may  Not
caused declining rate
form uniform dilation
confluent

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 1

 “honeycomb
pattern”
 cystic
lucency, with
white rings

ETIOLOGY
In here BRONCHIECTASIS is not the MAIN DIAGNOSIS it is a
CLINICAL MANIFESTATION
 Postinfectious conditions
 Like Tuberculosis
 Primary Immune Disorder
 Kartagener Syndrome
 Cystic Fibrosis
 Alpha-1-antitrypsin deficiency
 Heritable structural abnormalities
 Idiopathic inflammatory disorders
 Inhalational Accidents
 Chemical or ammonia inhalation
 Allergic Bronchopulmonary Aspergillosis/Mycosis
 Miscellaneous – HIV/ AIDS, Radiation injury
Causes: Generalized impairment of pulmonary defense
mechanism
PRIMARY CILIARY Kartagener’s syndrome
DYSKINESIA
-5-10% of bronchiectasis ( a -Situs inversus
typical entity, always expect -Bronchiectasis
this) -sinusitis
-Structural defects of dyein PRIMARY CILIARY
arms, radial spokes, & DYSKINESIA is also one of the
microtubules primary problem here
-Cilia becomes dyskinetic,
their coordinated, propulsive
action is diminished / Not
coordinated and not
functional
-Clinical effects:
Recurrent respiratory tract
infections (sinusitis, OM, &
bronchiectasis)
-Males are generally infertile

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 2

PATHOGENESIS PATHOLOGY

Destructive and Cartilage, muscle and elastic

inflammatory changes in
medium sized airways
GENERATION ZERO IS NOT
INVOLVED (THICKER
AIRWAYS NOT INVOLVED)
tissue are destroyed,
replaced by fibrosis (airway
becomes flat and fragile
resulting to a dilated airway)

The pathogenesis is best explained by the vicious cycle theory
as coined by Peter Cole.
This is an elaborated theory wherein coupled with hosts
underlying predisposition will bring about persistent, and
perpetuating inflammation and damaged to the airways.
It is also beneficial because it also gives you the management or
treatment for the patient depending on the step involved.
Harrisons 19th edition:
- Susceptibility to infection and poor mucociliary
clearance results in microbial colonization of the
bronchial tree.
- Some organisms, such as Pseudomonas aeruginosa,
exhibit a particular propensity for colonizing damaged
airways and evading host defense mechanisms.
- Impaired mucociliary clearance can result from
inherited conditions such as CF or dyskinetic cilia
syndrome, and it has been proposed that a single severe
infection (e.g., pneumonia caused by Bordetella
pertussis or Mycoplasma pneumoniae) can result in
significant airway damage and poor secretion
clearance.
- The 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
infectious/inflammatory cycle.
- Moreover, it has been proposed that mediators
released directly from bacteria can interfere with
mucociliary clearance.
**these what makes the lungs fragile with lessened capacity to
resist infection and inflammation.
CLINICAL MANIFESTATION
 Persistent or recurrent cough and purulent sputum
(typical presentation)
 Problem is how to differentiate it from COPD,
Chronic Bronchitis. The involvement of the
airways is the parameter, Bronchiectasis
involve the MEDIUM SIZED AIRWAYS. Others
usually involve the small and large airways.
 THICH TENACIOUS SPUTUM
 Hemoptysis (50-70%)
 Bleeding from friable, inflamed airway mucosa
(bronchial arteries)
 Good treatment strategy is just to
embolize the bronchial arteries
 Dyspnea, wheezing
 Recurrent infection

In addition to the clinical manifestation stated above,

the following symptoms listed in the diagram also exists.

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 3

 WET DRY  Sputum  Blood tests for
-productive cough of -Cough without sputum microscopy rarer immune
voluminous sputum production eosinophils and deficiencies
-Predominantly LUNG BASES -Usually involve ONLY the culture
and MID-PART OF THE LUNG UPPER LUNG FIELDS  Sputum smear
-If diffuse, LOWER LUNG is (minimal sputum present) and culture for
involved acid fast bacilli
 Skin tests (atopy,
EXACERBATIONS Aspergillus)
-Because BRONCHIECTASIS is an OBSTRUCTIVE DISEASE, that’s  High resolution,
why exacerbations is present thin section CT
 Increased sputum production scan
 24hr sputum volume  Sweat test (nasal
 Purulent sputum potential
 More bloody difference,
 The number of infective exacerbation per annum genotyping)
should be noted including frequency and nature of  Nasal
antibiotic usage. mucociliary
PHYSICAL EXAMINATIONS clearance (cilia
 Crackles (confined with one area of the lungs, persistent studies if
to a single area) abnormal)
 For pneumonia, crackles is diffused. Other
parts of the lungs are involved.
 May forever: HINDI SIYA AALIS SA LUGAR NA CHEST RADIOGRAPHY
TO. PERSISTENT SIYA SA ISANG LUGAR.
 Rhonchi
 Wheezes
 Clubbing (Not seen with COPD patients)
 Findings consistent with cor pulmonale and right
ventricular failure
 Due to complications of airflow limitation
CONSIDER THE DIAGNOSIS
“Persistent lung crackles on auscultation “
 Baseline chest x-ray
“Chronic or recurrent bronchitis or pneumonia”
 Sometimes patients has a normal chest x-ray
“Ashmatics for many years”
result
“Episodes of hemoptysis”
 So it is best to ask the patient if he/she has a
Recommendations for the investigation of:
previous CXR to compare
ALL patients SELECTED patients
 Dahil nga may forever, hindi aalis yung
 Chest  Fiberoptic
Bronchiectasis dun sa original niyang
radiograph bronchoscopy
lugar, so makikita mo yung mali sa CXR
(posterior  Barium swallow
sa dati and macocompare mo dun sa
anterior and (video
bago. Di niya iniwan yung lugar
lateral) fluoroscopy)
 Repeat CXR only if clinically indicated
 Sinus radiographs  Respiratory muscle
 Pulmonary function
function tests  Semen analysis
 Blood  Tests for
investigations associated
(CBC, ESR, CRP) conditions

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 4

  Should be done at least annually and quarterly for
immune deficiency or primary ciliary dyskinesia.
BRONCHOGRAPHY
 Gold standard but invasive (Lalabas to!)
 Instill Radioopaque, iodinated dye by catheter or
fiberoptic bronchoscopy
 Precaution for hypersensitivity, brochospasm and
anaphylaxis.
SWEAT CHLORIDE TEST
 To rule out Cystic Fibrosis
o CF is the most common cause of Bronchiectasis
Usually we see nonspecific findings in USA and industrialized countries
 “tram track” or tram lines
 “multiple ring shadows”
 Tubular or branched densities
High resolution CT scan (HRCT)
- Radiological investigation of choice to establish the diagnosis
-BEST NON-INVASIVE DIAGNOSTIC MODALITY! (Lalabas ulit to!)

You can see + cystic lucency BRONCHOSCOPY

CRITERIA FOR DIAGNOSIS  Localized disease to exclude proximal obstruction.
 Internal diameter of bronchus wider than the adjacent  Bronchoscopic sampling (not routine)
pulmonary artery  HRCT suggest atypical Mycobacteria & sputum culture
 Failure of bronchi to taper negative
 Bronchi being visualized in the outer 1 to 2 cm of the  during bronchoscopy, we appreciate the
lung fields friable and collapsibility of the airways.
Findings can suggest specific etiology
 ABPA
 Cystic fibrosis MANAGEMENT
 Immotile cilia syndrome GOALS OF TREATMENT
 Tubeculosis  Identify and treat underlying cause to prevent disease
 MOTT progression
SPUTUM STUDIES  Maintain or improve pulmonary function
 Should be obtained on all patients, to guide you in the  Reduce exacerbation
rational use of your antibiotics  Improvement of quality of life
 Specimen should reach the laboratory within 3 hours What to do?
 H. influenzae  Airway hygiene
 S. pneumoniae  Antimicrobial treatment
 S. aureus  Surgery
 P. aeruginosa  Others :
PULMONARY FUNCTION TEST  General supportive therapy
 To demonstrate airflow obstruction
 Identify presence of COPD
\

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 5

Airway Hygiene  MLA- host-defense modifying activity
 Tracheobronchial clearance techniques ANTI – ASPIRATION MEASURES
 Mechanical “valve” devices - Flutter, Acapella  Anti – GER management
, PEP  Improved deglutition
 Postural drainage and chest physiotherapy  Reducing gastric acid
 Therapeutic vest  Reduce food and liquid intake in the evening
 mechanical valve devices are designed to transmit  **chintac maneuver??? Diko to gets. To
agitating forces to loosen and help propel tenacious prevent the aspiration of the food that you
secretions. swallowed. If there is aspiration it can
 Postural drainage and cpt-prone head-down, damage the airway
trendelenberg, lateral decub postures are postions to ANTI-MICROBIAL THERAPY
help the patient cough out the sputum ANTIBIOTIC COMMENTS
Therapeutic vest  Episodic, targeted On the basis of C/S
 Pneumatically powered jacket antibiotics
producing high energy vibrating forces  Rotating antibiotic Week or month cycle
throughout the thorax therapy (Don’t use the same
 Proven in CF caused Bronchiectasis antibiotic continuously within
 May also be useful to other 3-6 months)
Bronchiectasis Quinolones are not routinely
 Fashionable used due to emergence of
resistance
 Initial empiric Prompt treatment for
AIRWAY HYGIENE followed by targeted exacerbation
antibiotics Best used is MACROLIDE
(Anti-Microbial and Increases
the defense to subsequent
infection)
 Inhaled antibiotics Tobramycin for
Pseudomonas
 Therapy for exotic Environmental
pathogens mycobacterial or fungi

 Long term oral antibiotics (3 or 6months)

 More than three exacerbations per year
 Mucus Mobilizing methods requiring antibiotic therapy
 Inhaled beta agonist (IBAs) and / or  Patients with fewer exacerbations that are
anticholinergic bronchodilators (ACBs) causing significant morbidity
 Hypertonic saline or mannitol inhalation  Quinolones should not be used until further
 Dornase alfa (Pulmozyme) studies are available.
 Efficient in controlling the secretion for Eradication algorithm for P. aeruginosa in adults
Cystic Fibrosis
 N-Acetylcysteine
 Anti-inflammatory airway management
 Systemic steroids
 Inhaled steroids
 Macrolide antibiotics
 NSAIDS
 Steroid alleviate mucosal edema and production of
inflammatory secretions.

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 6

If a patient is admitted due to Pseudomonas (fastidious
organism); after discharge or if the patient is demised. Don’t use
the room for the next 2 weeks.
SURGERY
Resectional Surgery Transplantation
-usually the lower lobes
-younger patients with
respiratory insufficiency.

Bronchial artery embolization – for massive hemoptysis (if

bleeding comes only from a particular lobe of the lung)

General Supportive Measures

 Vaccination – flu and Pneumococcal
 Smoking cessation
 Good nutrition & regular exercise
 Alpha – 1 – antitrypsin repletion
 Oxygen – for significant hypoxemia
 Methylxanthines
 Cromolyn, nedocromil,or leukotriene modifiers
 NO studies demonstrating efficacy

Hi to MIMI, NANCY and LHEEZA!

Happy Birthday Bea Loque!
SOURCES: 3A Recordings (Thanks Issa Caparros),
PPT, Harrison’s 19th Ed.

JOSEPH FRANCIS G. SAZON, RN (TEAM 3B-YATCH) 7