PULMONOLOGY, MED II - 3RD YEAR IMMUNE

DEFENSE OF RESPIRATORY SYSTEM Innate/Natural

SYSTEM
Adaptive/
( DR CONSTANTINO/ DR LEE TRANS, MIDTERMS February 2017) Immunity
Acquired
Immunity
(Nonspecific
(Specific
Responses)
Responses)
THE AIR WE BREATHE
Dr. Constantino: The air we breathe has a lot of particulate
matter, organic and inorganic, pollen, spores, bacteria, noxious FIRST LINE 2ND LINE 3RD LINE
fumes like tobacco smoke and formaldehyde, small particles
like carbon dioxide. All of these particulate matter can lead to
pulmonary disease. Specialized
1. Skin 1. Innate immune
2. Mucus cells Lymphocytes

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

PARTICLE SIZE membranes & 2. Inflammation 1. B cells ([produce
secretions 3. Complement antibodies)
3. Normal 4. Antimicrobial 2. T cells
Flora Substances a. Helper T cells
b. Killer T cells

NONSPECIFIC DEFENSE MECHANISM

Upper Airway: nose, larynx, trachea
(Inertial Impaction)
Lower Airways (Sedimentation – Gravitation)
Parenchyma: alveolar region
(Diffusion – Brownian movement)
 Anatomy of the respiratory system – If you analyze the
anatomy it has large airways branching into the much
smaller airways. It has several twists and turns in the
nasopharynx which would lead to inertial impaction.
 Clearance
 Secretions
 Cellular Defenses
 Biochemical Defenses
CONDUCTING AIRWAYS

Now particles more than 5 microns in diameter can pass through

the nose but they are usually impacted in the upper airway so Nares Nasal hairs
they are trapped there, inside the nose, the larynx or trachea. It Nasal turbinates
is because of inertial impaction. Because of the size and weight Nasal secretions
of the particle, once they encounter the turn or twist in the
respiratory tract, they stay there kung baga bumangga sa wall
Oropharynx
dumikit nalang doon. Epiglottis
Pharyngeal
Smaller particles 100nm to 1 micron, get trapped in the lower Lymphoid tissue
airways by sedimentation because of gravity. Larynx (Waldeyer’s Ring)

The very small particles, 1-100 nm and 1-5 microns may reach
the lung parenchyma to the alveoli and they remain there In the nose you have nasal hairs, turbinates and secretions that
because of diffusion or Brownian movement of smaller particles. are able to trap larger particles, those which are 10 microns or
more.
Now the immune system is comprised of innate or natural
immunity which consists of nonspecific responses and the In the oropharynx, you have the epiglottis, the pharyngeal and
adaptive/acquired immunity or specific responses. lymphoid tissue which form or act as protection for the
oropharynx before it goes into the larynx.
In Innate Immunity, the 1st line of defense would be the skin,
mucus membranes & secretions and normal flora. The 2nd line of NONSPECIFIC DEFENSE MECHANISM
defense would be innate immune cells, inflammation,
complement, and antimicrobial substances.
CLEARANCE SECRETIONS
- Nasal Clearance - Airway lining (mucus)
The 3rd line of defense from Adaptive/Acquired Immunity would - Tracheobronchial - Alveolar lining
be the specialized lymphocytes – B cells which produce the clearance (surfactant)
antibodies, T cells consisting of Helper T cells and Killer T cells. - Alveolar Clearance - Lysozyme
- Interferon
- Complement
In the nonspecific defense mechanisms, as mentioned, we have
the clearance mechanisms which consists of nasal clearance.
When you have something that goes into your nose, it stimulates
certain receptors and you sneeze. It may be a foreign object or
a particulate matter which could be potentially infectious. You
have tracheobronchial clearance, when something gets in your

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trachea or bronchia, you cough it out. You have alveolar Mucus – Sol layer; Gel layer. - Anyway, if you are able to
clearance which is dominated by alveolar macrophages. visualize this. You have 2 layers. The gel layer on top and below
that is the sol layer. In the sol layer, you have the cilia or hair-like
We have secretions in the airway lining consisting of mucus, in projections that move in attached forward motion propelling the
the alveolar lining (surfactant), lysozymes, interferon and gel layer towards the oropharynx.
complement.
(↑) Increase Mucociliary (↓) Decrease Mucociliary
CLEARANCE MECHANISM Clearance Clearance
 NASAL CLEARANCE - Beta agonist - Drying of airway
o Nose blowing & sneezing – in the presence of - Cholinergics - Cigarette smoke
irritant substances
- Substance P - Sulfure oxide (SO2)
o Mucociliary transport – consist of ciliated cells
- Methylxanthines - Nitrogen oxide (NO2)
 TRACHEOBRONCHIAL CLEARANCE
- Glyceryl guiacolate - Ozone/ Atropine

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

o Mucociliary clearance – again you have the
- Bromhexine - Narcotic agents
ciliated cells and the sol-gel layer. Ano ba yung
sol-gel layer? The ciliated cells are drained by the - Ambroxol - Codeine/ alcohol
liquid layer which is the sol layer and above that is - Barbiturates
the gel layer which consists of sticky mucus. - Topical anesthesia
Particulate matter can get trapped in the gel layer
and then because the sol layer is liquid, the cilia is IMPORTANT FUNCTIONS OF THE MUCUS BLANKET
able to move. They propel the stuck object out of 1. Facilitate ciliary action & remove unwanted particles
the airway into the nasopharynx to the oropharynx. 2. Provide a pool of antimicrobial proteins so the mucus
 ALVEOLAR CLEARANCE contains a lot of protective substances eg. Secretory IgA,
o Alveolar Macrophage Lysozyme, Interferons, Complement
o Mucociliary Escalator 3. Protect the epithelium from irritants, pH charge &
o Lymph Nodes enzymatic degradation e.g Alpha-1-anitrypsin – one
o Blood Stream example would be the presence of alpha-1-antitrypsin. It is
o Sequestration in the lung a blanket that cover the sensitive area
The alveolar macrophages and blood, in particulate matter or 4. Humidify inspired air – preventing the airways from drying
bacteria where later on they process into peptides and they go up so that definitely one of the conditions that decrease
into the lymph nodes in order for the elaboration of the mucociliary clearance is drying of airway diba
necessary antibodies.
SECRETIONS
Factors preventing pathogens from attaching to and  Mucus – sol & gel layer
colonizing upper respiratory tract surfaces:  Alveolar Surfactant
Factors that are protective.
o Opsonin
1. Decreased mucosal pH
o Bacteriocidal
2. Presence of naturally occurring bacteria & epithelial
o Decrease toxicity of inhaled particles
cell binding analogues, secretory IgA.
o Immunoglobulin
3. Constant desquamation of epithelial cells. – these are
 Lactoferrin
considered mechanism and they are removed from the
oropharynx.  Lysozyme
4. Nasopharynx colonized with nonpathogenic bacteria o Antimicrobial
that can interfere with attachments of pathogens to  Fibronectin
host cells by a variety of mechanisms – The presence of  Interferon
the nonpathogenic bacteria interferes with attachments of o Antiviral
pathogens, therefore they are not able to colonize in the o Antiproliferative
respiratory tract. This is the reason why people with o Antitumor
damaged respiratory tract mucosa, like patients with o Immunoregulatory
Bronchiectasis, for example, or other chronic lung diseases,  Immunoglobulins
they are prone to colonization by pathogenic bacteria like o IgA, IgG, IgM
Pseudomonas.
 Complement
o Co-factor
Factors responsible for interaction of nonspecific defenses
o Enhance vascular permeability
with specific immune responses in the lungs.
 Chemotactic factor
1. The size of the particle
2. The antigenic load
3. The physiochemical characteristics of the particle
which relate to the antigenicity, toxicity, and its
biologic properties (e.g type of virus, capsulated
bacteria, etc.)

MUCOCILIARY CLEARANCE
Consists of Cilia – above the ciliated cell and the

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COUGH CLEARANCE
Secreted substance are either bactericidal or
bacteriostatic
So you have a lot of secreted antimicrobial substances such as
lysozymes, lactoferrin and we will discuss them further in detail.
SURFACTANT
 Surfactant proteins A & D
 Bactericidal to certain pathogens
 Have instrinsic opsonizing property
 Decrease toxicity of inhaled particles
So they offer a lot of protection. What is the other function of
surfactant? They decrease surface tension and prevent collapse
of the alveoli.
LACTOFERRIN
 A 90,000 dalton iron-binding protein found in airway
secretion & as a constituent of neutrophil specific
granules
 Limits the availability of iron for bacterial metabolism
 Iron bound to Lactoferrin may play a role in the
generation of toxic oxygen species such as the
hydroxyl radical from H2O2 and molecular oxygen
The role of lactoferrin is to inhibit bacterial metabolism and
growth. It is also protective.
LYSOZYME
 Kills bacteria by destruction of the muramic acid, a
constituent of all bacterial cell wall
 More effective against gram (+) than gram (-)
bacteria
 It’s activity against gram (-) bacteria is enhanced in
the presence of antibody and activated
complement – so you need to have that in order to work
against gram (-) bacteria
 An important hydrolytic enzymes contained in
lysozomes, the membrane-bound intracellular
structures that participate in the killing of bacteria
after phagocytosis
FIBRONECTIN
 Produced by activated macrophages
Action of Fibronectin:
 Regulates cell adhesion & spreading of variety of
cells including fibroblast
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 Acts as a chemotactic factor so it attracts inflammatory
cells & works synergistically with other growth factors
including fibroblast replication
 Acts as opsonin for collagen, thus facilitates
clearance of partially degraded collagen – it cleans
up areas where you have bacterial activity
IMMUNOGLOBULINS
 IgA is the predominant immunoglobulin in the
nasopharynx and salivary secretions, exceeding IgG
concentration by about 3:1. – so you have more IgA in
the upper airways
CAYRAPF – MED II, PULMONOLOGY - FEBRUARY 2017, 2ND SEMESTER
 The concentrations of IgA & IgG are about equal in
the large airways, but the proportion of IgG increases
distally in the conducting airways. – as you go deeper
into the lung, the ratio of IgA to IgG becomes different. You
have more IgG in the lower airways.
 The concentration of IgG exceeds IgA by about 4:1
at the alveolar level. – IgA in the upper airways. IgG
in the lower airways!
 Bacteria more effectively phagocytosed by alveolar
macrophages when coated or opsonized with IgG
than with IgA. Anyway most of the phagocytosis of
bacteria occurs in the lower airways in the alveoli.
Immunoglobulin A (IgA)
1. Bind inhaled antigens
2. Neutralize viruses
3. Interferes with adherence of some bacteria to
the respiratory mucosa – offers a lot of mechanisms
for protection
4. IgA does not fix complement and does not serve
as an effective opsonin in promoting
phagocytosis by neutrophils & macrophages
Remember what we have talked about earlier. It is IgG that plays
the role with phagocytosis.
Immunoglobulin G (IgG)
 The major immunoglobulin in the lower airway and
alveolar surface – Remember that the ratio is 4:1.
 It fixes complement and opsonizes bacteria to
increase the efficiency of phagocytosis by
macrophages and neutrophils.
Immunoglobulin M (IgM)
It does not usually play a big role in lung defense. We have
normally decreased amounts in the airway fluids but during
inflammatory responses in the lung, the level of IgM increases.
 Normally only trace amounts is found in the airway
fluids, but rises during inflammatory responses in the
lung.
COMPLEMENTS
 Act as co-factor
 Enhancement of vascular permeability and
chemotaxis of PMNs – so much so that they are
participative in the inflammatory response
 Main biological active product of the complement
system
 C3a – anaphylatoxin activity
 C3b – promote phagocytosis
 C5a – anaphylatoxin & chemotactic activity
- Increases adhesiveness of PMN
 - Promotes release of PMN lysosome
 C5b – membrane attack
NONSPECIFIC DEFENSE MECHANISM
 Cellular Defenses
- Non-phagocytic
 Airway Epithelium
 Terminal Respiratory Epithelium
- Phagocytic
 Blood Phagocytes (PMN, Monocytes)
 Tissue Phagocytes (Alveolar Macrophages)
 Biochemical Defenses
- Anti-proteolytic Enzyme
- Antioxidants
AIRWAY EPITHELIUM
 Acts as barrier to dissemination of microorganisms
and serve important immune functions – acts like a
wall to prevent entry of microorganisms into the respiratory
mucosa
 Produces antimicrobial molecules
 Participates in upregulation of inflammation by:
Recruitment of phagocytic cells
Chemokine production:
1. Interleukin 8 (IL-8)
2. Macrophage inflammatory protein
3. Granulocyte-macrophage colony stimulating
factor
4. By-product of arachidonic acid and cascade
 Regulate expression of endothelium adhesion
molecules – which are all protective
DENDRITIC CELL NETWORK
 Dendritic cells are antigen-presenting cells that
constitutively express a high level of MHC (Class II)
antigen. When the alveolar macrophage engulf the
bacteria, they are processed into peptides which are
brought by dendritic cells to the lymphoid tissue.
 Reside in the airways with branching processes that
extend in the plane of the epithelial basement
membrane forming a network optimally situated to
sample inhaled antigen
 Highly efficient antigen-presenting cells especially
after maturation under the influence of TNF-and GM-
CSF
 Considered the most potent antigen-presenting cells
PINAPANSIN NIYO BA YUNG
MGA NAKA-BOLD TYPE?
BAKIT SILA NAKA-BOLD TYPE?
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Epithelial
Dendritic cells,
receive viral & Immune
cells, bacterial cells,
protect information to secrete Cilia stroke
airway prime the IgA the mucus
Goblet cells,
immune system upward
produce
mucus
CAYRAPF – MED II, PULMONOLOGY - FEBRUARY 2017, 2ND SEMESTER
Illustration of the Upper Airway Wall
Note the defense systems: mucus layer, ciliae to sweep the mucus out
and secretory IgA to immobilize bacteria and viruses.
MACROPHAGES
Most important cell in lung defense
 Primary defense of the lungs
 Physically remove from direct contact with pulmonary
tissue all particulate matter that enters the peripheral
airways, particularly bacteria, organic and non-
organic dust particles. – They are removed by Alveolar
macrophages. Even TB bacilli. How do you get TB infection?
Diba by inhalation. After inhalation of the TB bacilli where
does it go? Where in the lungs? They go initially into the lower
lobes. Because that is the most well ventilated. If you are
immunodeficient at that point, may Diabetes ka or HIV, you
may develop TB Pneumonia. Even in children may Ghon
complex/lesion nasa lower lobes yun meron pang
Pneumonitis. And then, in the alveoli, the TB bacilli are
engulfed by the alveolar macrophages. Then, they are
brought to the lymph nodes and disseminate in the blood
stream. Eventually they end up in the apex of the lung
because of what? High oxygen tension. So now, it depends
on what part of the TB infection you were immunodeficient.
Let’s say you have TB in the lymph nodes and you are
immunodeficient, you have TB of the lymph nodes. If you are
immunodeficient at the time it is in the bloodstream, you will
have Disseminated TB. Yung Chest xray iba diba usually TB
may apical lesion, you have cavitations but then if you have
disseminated TB, you have Biliary TB, in the bloodstream, and
then you have TB of the other organs, TB of the liver, spleen,
pericardium. TB can be anywhere. Ang pwede lang hindi
magka-TB kasi yung buhok. Usually apical TB is the last stage,
it’s a retroascending infection, it means you had been
infected with TB for a long time. Hindi siya descent pag nasa
apex kasi yun na yung pinakalast stage. It stays there for
several years or months until the time you have weakened
immune responses, it becomes active. Usually in adult TB, you
have TB of the lymph nodes. That’s why in 50% of cases who
have TB effusion, you do not have apical infiltrates. So when
you have pleural effusion and you do not have apical
infiltrates, it does not rule out TB as the cause of your effusion.
ALVEOLAR MACROPHAGES
Represent 85% of the cells – remember that it is the most  Hydrogen peroxide – damage cell by causing strand
important cell breaks in nuclear DNA
 Constitute the primary defense of the lungs  Hydroxyl radicals – highly reactive, can contribute to
 Participate in most defenses not only by phagocytosis lipid peroxidation, DNA damage and protein
but also by secreting other substances that damage
participate in lung defense
PROTEASE INHIBITOR
FUNCTIONS OF PULMONARY MACROPHAGES  To restrain the potentially destructive consequence
Defensive Functions of inflammation by deactivating newly released
1. Microbicidal activity & bacteriostatic – kill or stabilize proteolytic enzymes
the bacteria  Alpha-1-antitrypsin
2. Phagocytosis of particulate & insoluble substances - Quantitatively the most important – due to large

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

including antigen-antibody complexes amounts.
3. Uptake, localization & processing of antigen - Inhibits lytic enzymes released from leukocytes &
4. Degradation of antigen & particles macrophages, plasmin, plasma thromboplastin
5. Synthesis of: antecedent and kallikrein – preventing severe
- Immunoregulatory substances inflammatory damage
- Complement compounds
- Chemotactic factors ANTIOXIDANT ENZYMES
- Tumor-inhibiting factors  Superoxide dismutase
It has a lot of functions that’s why it is the most important cell in  Catalase
lung defense.  Glutathione peroxidase
Non-defensive Functions  NADPH
1. Non-microbial scavenging  Cytochrome C reductase
2. Synthesis of:  Oxidant free radical scavengers
- Arachidonic acid metabolites - Vitamin E
- Platelet activating factors - Beta carotene
- Fibroblast activating factors - Vitamin C
- Enzyme inhibitors
- Binding proteins OXIDANT INHIBITORS
 Superoxide dismutase convert superoxide anion into
NONSPECIFIC DEFENSE MECHANISM H2O2 a less reactive molecule
 BIOCHEMICAL DEFENSE  Catalase catalyzes the degradation of H2O2 into
- Anti-proteolytic Enzymes water and O2 – it becomes harmless
- Antioxidants  Reduced glutathione contributes to degradation of
 Protease inhibitors large molecule hydroperoxide and lipid peroxides
- Alpha-1-antitrypsin – inhibits protease which is a
dendritic enzyme that destroys the elastic framework of DEFENSES OF THE RESPIRATORY TRACT
the lung. Sometimes alpha-1-antitrypsin is also known as
anti-elastase. That’s why if you have a deficiency of Upper airways & -Anatomic barriers
alpha-1-antitrypsin, you develop an emphysema-like Bronchi - Cough
disease of the lungs
- Mucocillary apparatus
 Oxidant inhibitors - Airway epithelium
- Superoxide Dismutase - Secretory IgA
- Catalase - Dendritic cell network
- Glutathione Peroxidase - Lymphoid structure
- NADPH
- Cytochrome C Reductase Alveolar Spaces - Alveolar macrophages
- Alpha-tocopherol - Immunoglobulins &
- Ascorbic acid Opsonins
These oxidant inhibitors reduce the action of oxidants relieving - Lymphocyte-mediated
oxidative stress. Immunity
OXIDANTS - Neutrophils &
 A double-edged sword: Eosinophils
 1st – the direct cytotoxicity of the reactant – can
destroy cells This is a review of the lung defense. You have the upper airways
 2nd – their ability to inactivate alpha-1-antitrypsin – and bronchi, wherein you have anatomic barriers, the structure
can overcome alpha-1-anitrypsin that’s the reason why of the upper airway itself – the presence of numerous branches,
when you have alpha-1-anitrypsin imbalance, you develop presence of turbinates, nasal hairs, form anatomic barriers which
lung disease that will lead to inertial impaction of inhaled particulate matter.
 Superoxide anion – damage enzyme and can attack You have cough reflex, mucociliary apparatus which sweeps
inhaled particulate matter in the gel layer, it sweeps them up
unsaturated fatty acids causing lipid peroxidation
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toward the oropharynx wherein they are coughed out or
sneezed out. You have the airway epithelium which acts as a
barrier and secretes substances that are protective. Secretory
IgA, remember, that it is the one that is in large amounts in the
upper airway with 3:1 ratio. The dendritic cell network and
lymphoid structure which is connected to the dendritic cell
network.
ADAPTIVE IMMUNITY
I don’t think we need to go into detail on this. Maalala niyo pa ba iyan? You
have to look at your basics especially if you don’t know the right answer, in
order for you to guess the correct answer. Do not forget your basics
because this is your foundation of what you will learn later on in clinical
medicine. That’s how I survived medicine. I was able to guess intelligently.

In the alveolar spaces, you have the alveolar macrophages

which is the most important cell in lung defense, the presence of
immunoglobulins & opsonins, lymphocyte-mediated immunity,
and neutrophils & eosinophils.

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

You are familiar with these cells in the 2nd year.

COMPLEMENT SYSTEM

DEFECTS IN HOST DEFENSE THAT CAN BE ASSOCIATED TO

RESPIRATORY INFECTION

EXPOSURE TO VIRULENT
ORGANISM OR TO LARGE INOCULUM

You have the complement system which arises from the

interplay of your neutrophils, monocytes, mast cells,
macrophages, dendritic cells, B cells & T cells. INHALED OR ASPIRATED
IN THE LUNG
You have your innate immunity produced by opsonization, lysis
of pathogens, chemotaxis, inflammation, cell activation.

You have adaptive immunity produced by augmentation of

antibody response, promotion of T-cell response, elimination of ILLNESS
self-reactive B cells, enhancement of immunologic memory.

Also, the disposal system which consists primarily of the

DEFICIENCY OR
clearance of immune complexes and apoptotic cells, yung mga MALFUNCTION OF
dead or necrotic cells. HOST DEFENSE SYSTEM

RECURRENT OR
CHRONIC INFECTIONS

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Exposure to virulent organism or to large inoculum. If it is a virulent Surfactant Decreased Loss of opsonization activity;
microorganism, you will only need a small amount to develop (protein) synthesis; Acute alveolar collapse (atelectasis)
infection. If it is avirulent, you need a large amount to get infection. lung injury
Alveolar Subtle effects Propensity for intracellular
It can either be inhaled or aspirated in the lungs and develop
macrophages from microbes and Legionella
illness. If you have deficiency or malfunction of host defense immunosuppres infections; poor containment of
system, you get recurrent or chronic infections. sion; Mycobacteria spp.
Cannot kill
COMPONENT POSSIBLE RELATED IMPACT OR INFECTIONS intracellular
IMPAIRMENT microbes
DEFECT PMN Absent because Poor inflammatory response,
Conducting Airways granulocytes of immune- associated with gram-negative
Mechanical Bypassing Aspiration, direct aerosol or suppression; baciliary infection and fungi
barriers barriers with an microbes into airway – (ex. intrinsic defects (Aspergillus)
(larynx, etc.) E.T or Patient w/ ET tube, wherein he of motility; lack

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

tracheostomy can aspirate to the junction of chemokine
between the larynx & the stimulus
endotracheal tube wherein you Augmenting mechanisms
have the cuff & it may be Inhibition of Immuno- Inadequate SigA or IgG
deflated or pt has a immune suppression antibody available (more
tracheostomy tube and here responses susceptible to viral,
comes the respiratory therapist. (humoral muycoplasmal, and bacterial
Usually, the tubes in the antibody and infections)
respirator develop fluid inside & cellular
you have to drain that. The immunity)
proper way to drain that is by Generation of Generally Inadequate inflammatory
draining through the upper end inflammatory reflects status response, recurrent infection
yung distal end. Sometimes the response and supply of
R.T drains from the proximal end, (influx of PMN PMN
pag dinrain mo dun yung fluid na granulocytes, granulocytes;
may bacteria ppunta dun pag eosinophils, impaired
kinabit mo ttulak ng respirator lymphocytes, adherence to
yun papasok yung bacteria. and fluid endothelium
Mucociliary Intrinsic Stagnant secretions (not able to components)
Clearance structural defect move and cough it out so you
In the immunosuppression is due to the lung trying to protect itself
(cough) in cilia; ciliotoxic get recurrent infections like
infections bronchiectasis), coughing,
from the presence of bacterial infection. Actually when you have
bronchiectasis, sinusitis, Pneumonia, it is actually a reflection of the inflammatory response
pneumonitis (Ano yung of the lung. That’s why you have several stages.
syndrome na may sinusitis at
bronchiectasis tapos may siTus HOST DEFENSES IN THE APPROACH TO PATIENTS WITH
inverSus? SINUSITIS,
BRONCHIECTASIS, SITUS
PULMONARY DISEASE
INVERSUS = KARTAGENER DIFFERENTIAL DIAGNOSIS FOR HOST DEFICIENCY
SYNDROME! Ciliary immotile MANIFESTED IN LATER LIFE:
syndrome may abnormal cilia,  Cystic Fibrosis (adult onset) – secretions leading to
recurrent sinusitis, & infertility
kasi the sperm has abnormal
recurrent Pneumonia
ciliary movement. Hindi  Selective absence of IgG subclass immunoglobulins
marunong lumangoy nalulunod.  Structural ciliary defects – Like what I mentioned earlier,
Usually these pts don’t have the Kartagener syndrome
children.
Bronchoconstri Hyperactive Poor removal of secretions,
 IgA deficiency
ction & airways, asthma excessive secretions  Recurrent sinopulmonary infections – important clue
mucosal
edema APPROACH
Local IgA deficiency Sinopulmonary infections;
Immunoglobuli or functional abnormal colonizationg with
1. Detailed History – any affected siblings, infertility,
n coating- deficiency from bacteria striking change in respiratory health in respiratory
secretory IgA breakfown by detail based on acquired abnormality or
bacterial IgA1
likely/sudden – check also for history of infertility like I
proteases
mentioned before if you have ciliary dyskinesia it can
Iron-coating Iron deficiency May not inhibit certain bacteria
proteins (like (Pseudomonas, Legionella) also involve the spermatozoa. Male patients recurrent
Transferrin or sinopulmonary infections with infertility or no chidren may
Lactoferrin) have possibility of ciliary dyskinesia syndrome
Alveolar space 2. Preliminary screening tests
Other Acquired Sinopulmonary infections; a. CBC – check for possibility of bacterial infection. If
immunoglobuli hypogamma- Pneumonia with encapsulated
n classes globulinemia, bacteria
it is normal, it may be viral or tuberculum.
(opsonic IgG) Selective IgG2 & b. Quantitative serum immunoglobulins
IgG4 deficiency c. Pulmonary function tests (even if CXR is
Alternative C3 & C5 Recurrent infection possible normal) sometimes interstitial lung diseases
complement deficiency
cannot be seen on ordinary CXR, they will only be
pathway
activation seen by chest CT scan.
d. Cultures of respiratory secretions to identify
etiology

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 e. Electrolyte analysis in sweat – For sweat
Chloride Test of Cystic Fibrosis
Secondary Level Screening Tests
- More expensive and complicated test
- Contemplation of Subclassic IgG, Secretory IgA
- Parotid and fluid nasal wash
- Subtyping of blood lymphocytes
- Measurement of Antibody response to protein and
polysaccharide antigen
- Search of genetic mutations of cystic fibrosis
transmembrane conductance regulator
- Secondary screening tests are not available here.
 Mucoid strains of Ps. Aeuroginosa and elevated

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

sweat chloride – seen in CF
f. Assessment of ciliary clearance with
aerosolized, isotopic tracer
g. Nasal mucosal biopsy for electron
microscopic ultrastructural analaysis of ciliary
dyskinesia syndrome
h. Sperm motility tests
i. CT scan for documentation of bronchiectasis
3. Thorough evaluation by Otolaryngologist – check
for recurrent sinusitis, otitis media, nasal polyps –
because these defects lead to recurrent Pneumonia and
eventually Bronchiectasis
 Certain forms of Pneumonia may point to possible
deficiencies in lung cells such as AM, lymphocytes or
PMNs
 Lack of opsonic antibody can promote infections
with encapsulated bacteria such as Pneumococci
 Infection with Legionella pneumophilia reflects
abnormal lymphocyte function and cell-mediated
immunity – after infection the host develops specific
IgM and IgG serum antibodies which do not create a
lytic state that is sufficient to kill the bacteria
 AIDS – example of defect at the level of the
lymphocytes, in which the host is infected with HIV
that destroys CD4 TH lymphocytes
 AIDS patients – experience recurrent respiratory
infections with diverse organisms:
i. Viruses (cytomegalovrisu or herpes simplex)
ii. Pneumocystic carinii
iii. Mycobacterium tuberculosis
iv. M. Avium – intracellulare
v. Cryptococcus specie
vi. Toxoplasma gondii
vii. Legionella
4. CD4 deficiency in lungs of patients with AIDS
compromises the activation of AM to kill or control
microbes – causing impressive defect in cell-
mediated immunity

INTACT & ADEQUATE LUNG DEFENSE PROTECT AGAINST

DISEASES
Any defect will lead to Pulmonary Disease.

-LIGHT-
Please do not rely on this. Double check for any mistakes. Read
the book and study well. God bless, doctors! Thank you.
CAyraPF

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