Diffuse Pulmonary Diseases
Diffuse
Pulmonary
Diseases
Diffuse
Restrictive
Pulmonary
Diseases
Diffuse
Obstructive
Pulmonary
Diseases
Insterstitial
Pulmonary
Diseases
Chest Wall
Disorder
Emphysema
Chronic
Bronchitis
Asthma
1. Severe Obesity
2. Neuromuscular
Disorder
Chronic
Acute
Centriacinar
Panacinar
3. Disease of the pleura
Acute
Respiratory
Distress
Syndrome
Occupational &
Environmental
Exposure
Pneumoconiosis
(Inorganic)
Drug &
Treatment
Related
Hypersensitivity
Pneumonitis
(Organic)
Immune Related
Miscellaneous
Post ARDS
Idiopathic
Pulmonary
Fibrosis
Distal Acinar
Irregular
Bronchiectasis
Diffuse Obstructive Pulmonary Diseases
Characteristics
 Normal FVC
 ↓FEV1
 ↓FEV1/FVC
Definition

Limitation of airways

↑resistance caused by
o Partial or complete obstrcution of airways
Diseases
Emphysema
Permanent dilatation of airspace distal to the the terminal
bronchiole
With destruction of the alveoli wall
Categorized into
o
Centriacinar
o
Panacinar

Definition



o
Types
Centriacinar



Macroscopic
o





o
o


Asthma


Airways are
Swollen
Hypereamic
Covered by mucopurulent/
mucinous secretion
Copious sputum

Tracheal glands can be
Hyperplasia
Hypertrophy
Goblet cells can be
Hyperplasia
Metaplasia
Thickening of the submucosal gland
compared to that of the bronchial wall
Measured through Reid index

Normal – 0.4
Marked number of inflammatory cells,
neutrophilia can also increased
Fibrosis may lead to a complete
obstruction to the airways
This is called Bronchiolitis Obliterans



Loss and thinning of alveolar walls
Consequent enlargement of airspaces
Extensive loss of pulmonary capillaries
along the way

o
o

o
o
o
Microscopic


o
o
o
o

o
o
o
o
Causes

Centriacinar
o Heavy smoker

Panacinar
o Congenital Alpha-1 Antitrypsin
Causes

Chronic cigarette smoking

Chronic exposure to air
pollutants
o

Mucus plugs contain
Curschmann Spirals

Whorls of shed epithelium
Charcot-Leyden Crystals

Crystalloids from Eosinophils
proteins
Eosinophils
Airways remodelling, which include
Thickening of basement membrane of
the bronchial epithelium
Edematous with inflammatory cells
infiltrate

Prominence appearance of

Eosinophils

Mast cells
Increased size of Submucosal Glands
Hypertrophy of Bronchial smooth
muscle cells

Pneumonia
Lung abscess
Empyema




TH2 and IgE mediated
immunologic reaction to

Environmental allergens
Non-atopic Asthma

o



Lungs were overdistended due
to hyperinflation
Small areas of Atelectasis
Occlusion of Bronchi and
Bronchioles by thick tenacious
mucus plugs
Causes

Atopic Asthma
o
o
Complications

Permanent dilatation of
o
Bronchi
o
Bronchiole
Due to the destruction of
o
Elastic tissues
o
Smooth muscle cells

Involved the LOWER LOBE of
BILATERAL lungs
Usually affect the more distal
bronchi and bronchioles
almost near to the pleura
The airways may dilate to
almost 4 times of normal
diameter
o
May extend until the
level of pleura


Usually happens at the LOWER LOBE
The entire respiratory acinus, from respiratory
bronchiole to alveoli, is expanded
Lungs appear

Pale in color

Highly voluminous

The lungs were so extended, it obsecured
the heart

Additional Infos
Bronchiectasis
Chronic inflammatory disorder of airways
Characterized by early morning and
night
o
Wheezing
o
Breathlessness
o
Chest tightness
o
Cough
Panacinar


Morphology
Usually happens at the UPPER LOBE
The respiratory bronchiole (proximal and central
part of the acinus) is expanded. The distal acinus
or alveoli are unchanged
Lungs appear

Deeper pink

Less voluminous unless in advanced stage
Chronic Bronchitis
Persistent productive cough for at least
o
3 consequtive months
o
In at least 2 consequtive years

Viral infections
Inhaled pollutants
***less clear
Septiceamia
Cor Pulmonale
Metastatic Cerebral Abscess
Secondary Amyloidosis
o
o



In full-blown active case
Massive infiltration of acute and
chronic inflammatory cells
Happens within the wall of

Bronchi

Bronchiole
Desquamation of epithelium may lead
to extensive tissue ulceration and
necrosis
Various pathogenic flora harbor the
involved bronchi
Necrosis leads to formation of lung
abscess
Causes

Obstruction
o
Tumors
o
Foreign bodies

Congenital Abnormalities
o
Cystic Fibrosis

Due to abnormal secretion of
viscid mucus
o
Immunoglobulin deficiencies

Repeated bacterial infections
o
Kartagener Syndrome

Autosomal recessive

Impaired function of cilia
Diseases
Age
Dypsnea
Cough
Infections
Respiratory
Insufficiency
Cor-Pulmonale
Airway Resistance
Elastic Recoil
Chest Radiograph
Differences Between Chronic Bronchitis and Emphysema
Chronic Bronchitis
Emphysema
40-45 years old
50-75 years old
 Mild
 Happens late
 Early onset
 Copious
Common
 Severe
 Happens early
 Late onset
 Scanty
Occasional
Repeated
Terminal
Common
Rare, happens at the terminal stage
Increased
Normal or slightly increased
Normal
Abnormal
Large heart
Small heart
Blue Bloater


Appearance

Due to hypersecretion of mucus, the airways tend
to block, there is NO damage of blood vessels
Due to obstruction, compensatory mechanism
takes place
o Increased perfusion through increasing the
Cardiac Output
o Reduced in Ventilation due to obstructed
airways
o Leading to ↓V/P ratio
o Hence, reduce in ventilation make the
compensatory mechanism less efficient
 Oxygen cant be pass to the body
leads to Cyanosis (Blue)
Other than tend to be obese, the lungs are
hyperinflated, therefore patients appeared Bloated
Pink Puffer



Permanent dilatation of alveoli walls with damage
to blood vessels
Due to less pulmonary compliance, compensatory
mechanism takes part
o Increased in ventilation, the patient appears
gasping for air (Puffer)
o The perfusion tends to increased significantly
eventhought the some of the blood vessels
are destroyed
o Hence, normal V/P ratio
o No Cyanosis
Increase in ventilation will lead to increase in
mechanical stress
o Therefore the upper abdominal wall appears
pinkish
Diffuse Restrictive Pulmonary Diseases
Characteristics
 ↓FVC or Normal FVC
 Proportionate ↓FEV1
 Normal FEV1/FVC
Definition

↓expension of lungs parenchyma

Accompanied by ↓lung capacity
Pneumoconiosis


Definition
1.
Factors Contributing to the
Extent of Pneumoconiosis
2.
3.
Is one types of diffuse restrictive diseases
It is an occupational lung diseases
4.
Amount of dust retain in the lung and airway
a.
Degree of air pollution
b.
Duration of exposure
Size and shape of particles
a.
Minute particles may
i. Reach the terminal airways and alveoli
ii. Settle in their lining to evoke inflammatory response
Additional effects of other irritants
a.
Tobacco smoking magnifies the effect of Asbestos
Particles solubility and physiochemical reactivity
a.
Smaller particle
i. Tends to dissolve and reach toxic level in the pulmonary fluid
ii. May to lead to an acute lung injuries
b.
Larger particle
i. Due to its size, larger particle resists dissolution
ii. Instead it will remain inside the lung parenhcyma and lead to chronic lung
injury
Types of Pneumoconiosis
Diseases
Coal Worker’s Pneumoconiosis (CWP)

Definition


Macroscopic

Morphology


Simple CWP
o
Coal macule
o
Nodule
o
These are located primarily at

UPPER LOBE

UPPER PART OF THE LOWER LOBE
Complicated CWP
o
Intensely blackened scar

Assymptomatic Anthracosis
Linear streak
Aggregate of black anthracotic pigments
Simple CWP
o
The nodules contain

Dust-laden macrophages adjacent to respiratory bronchioles

Delicate collagenous networks
o
Panacinar emphysema may occur if the dilatation of alveoli is
extensive
Complicated CWP
o
Center lesions often necrotic due to ischemia
o
The lesions are packed with

Collagen

Pigment
Complicated CWP with PMF may have increased
o
Pulmonary dysfunction
o
Pulmonary hypertension
o
Cor-Pulmonale
Pulmonary dysfunction

Cor-Pulmonale
Pulmonary hypertension

COPD independent of smoking

o
o

Microscopic


Clinical Features
Complications


Silicosis
Type of Pneumoconiosis due to prolonged breathing in dust from
o
Coal
o
Graphite
o
Man-made carbon
Spectrums of CWP
o
Asymptomatic Anthracosis
o
Simple CWP

With little/no pulmonary dysfunction
o
Complicated CWP/ Progressive Massive Fibrosis (PMF)

<10% of simple CWP proceed to PMF


Type of Pneumoconiosis due to inhalation of
o
Crystalline Silicone Dioxide (Silica)
Usually present after decades of exposure
o
Presented as Nodular Fibrosing
Pneumoconiosis
Nevertheless, heavy expsure in a short period of
time may lead to an Acute Silicosis
Asbestosis


**The most prevalent type of Pneumoconiosis
Silicotic nodule
o
Early stage
The nodules are

Tiny

Barely palpable

Discrete

Pale-to-black

Located at the UPPER LOBE
Progressive stage

The nodules may

Coalesce

Become hard

Collagenous scar


o






Type of Pneumoconiosis due to 20 years or more inhalation of
o
Asbestos
Asbestos exposure is linked with
o
Asbestosis (discussed here)
o
Localized fibrous plaques
o
Pleural effusions
o
Cancers

Lung carcinoma

Mesotheliomas

Laryngeal carcinoma

Colon cancer
Massive fibrosis present
o
Begins at the LOWER LOBE and SUBPLEURA
o
Extends to the UPPER LOBE
Contraction of the tissue distorts the normal tissue
architecture of the lungs
Visceral pleura may also become fibrotic
o
The thickening stick with the anterior chest wall
o
Lead to narrowing of arteries and arterioles, leading
to

Pulmonary hypertension

Co-Pulmonale
Silicotic nodules are
o
Concentric layers of Hyalinized
collagen
o
Surrounded by dense capsule of
condensed collagen
o
With or without necrosis
Under Polarized Microscope
o
Birefringent silica particles

Most workers are assymptomatic, accidental
finding upon X-ray
Shortness of breathe becomes apparent when
progressive massive fibrosis takes place
Silicotuberculosis
o
Silica may inhibit the activity of
macrophages



Dypsnea
Productive cough
Clubbing of fingers in the late stage


Respiratory failure
Cor-Pulmonale

Asbestos bodies
o
Golden brown
o
Fusiform/ beaded rods
o
Translucent center
o
Asbestos fibers coated with iron-containing
proteinaceous materials
Air spaces become capsulated with fibrotic tissue
leading to Honeycomb appearance
Disease
Sarcoidosis
Granulomatous Diffuse Restrictive Pulmonary Disease
Definition and Epidemiology
Pathogenesis
Clinical Features
Definition
 Chronic inflammatory  65-70% patient heal
 Systemic granulomatous disease
regulation is
without
 Present with various clinical
disorganized by
complication, either
presentation
which
o Spontaneously
o Bilateral Hilar Lympadenopathy
o ↑intraalveolar and
o With Steroid
in 95% of cases
interstitial CD4+ T
therapy
o Followed by
cells

10-15%
patients
 Lesion over Periorbital region
o ↑TH1 cytokines
succumb to
 Crops of numerous Papules
 IL-2
o Pulmonary fibrosis
on the skin
 TNF
o Cor-Pulmonale
 Increased release of
o Die due to
Epidemiology
 Adults younger than 40 years old
other cytokines
 Cardiac
 Common in
which favor
damage
o Danish
recruitment of
 CNS damage
o Swedish
Monocytes
o African American
 This has impinged
injury on tissue
Disease
Idiopathic Pulmonary
Fibrosis (IPF)
Usual Interstitial Pneumonia
(UIP)
Morphology
 Formation of multiple
well-formed Noncaseating Granulomas
 The granulomas can
be
o Schaumann Bodies
 Laminated
concretions
composed of
 Calcium
 Proteins
o Asteroid Bodies
 Stellate inclusion
within Giant Cells
** these inclusion
bodies are NOT
pathognomonic of
Sarcoidosis
Miscellaneous Diffuse Restrictive Pulmonary Disease
Definition and Epidemiology
Clinical Features
Definition
Early symptoms
 Clinicopathologic syndrome with
 Increasing dypsnea on exertion
characteristic
 Dry cough
o Radiological features
Late symptoms
o Pathological features
 Hypoxeamia
o Clinical features
 Cyanosis
Epidemiology
 Clubbing of fingers
 40-70 years old
It develops insidiously, the patients usually
 Gradually deteriorate despite of treatment
 Mean survival rate – 3years or less
 Lungs transplant is the only definitive therapy
Acute Diffuse Restrictive Pulmonary Disease
Disease
Etiology
Pathogenesis
Direct Lung Injury
o Common causes
 Pneumonia
 Aspiration of
gastric content
o Uncommon causes
 Pulmonary
contusion
 Fat embolism
 Near drowning
 Inhalational injury
 Indirect Lung Injury
o Common causes
 Sepsis
 Severe trauma
with shock
o Uncommon causes
 Cardiopulmonary
bypass
 Acute
pancreatitis
 Drug overdosing
 Transfusion of
blood product
1. Acute assult leads to increase
production of
a. IL-8
i. Neutrophils
1. Activation
2. Chemotaxis
b. IL-1 and TNF
i. Neutrophils
1. Activation
2. Chemotaxis
ii. Endothelial activation
c. Pulmonary microvascular
sequestration
2. These will subsequently lead to increase
number of Neutrophils leading to
increase secretion of
a. Leukotriene
b. Oxidants
c. Proteases
3. Release of these chemical may lead to
a. Damage to the epithelium
b. Maintainance of inflammatory
damage
4. Nevertheless, it is counteract by antiinflammatory substances such as
a. Anti-oxidant
b. Anti-protease
c. IL-10
5. Imbalance of this regulation may lead to
an acute damage to the lung

Acute
Respiratory
Distress
Syndrome
(ARDS)
Morphology
Macroscopic
Microscopic
Lungs appear
Acute stage
 Dark red
 Capillary congestion
 Firm
 Necrosis of alveolar
 Airless
epithelial cells
 Heavy
 Interstitial and
Intraalveolar
o Edema
o Heamorrhage
 Present of Hyaline
membrane lining the
alveolar ducts
 Infiltration of
inflammatory cells
Organizing stage
 Marked proliferation
of Type 2
Pneumocytes
 Fibrinous exudation
leads to alveolar
fibrosis
 Marked thickening of
alveolar wall
o Due to proliferation
of Interstitial cells
 Deposition of
collagenous fibers