Restrictive lung disease I

Question 1

can be due to

Answer 1

intrinsic (parenchymal) or extrinsic disease

Question 2

what is seen with restrictive disease

Answer 2

***Reduced total lung volume, resting volume, or
vital capacity
-FRC is normal (increased in obstructive
diseases)
-FEV1 is normal (FEV1/FVC is decreased in
obstructive diseases)

Question 3

Pneumoconiosis

Answer 3

-A group of diseases caused by inhalation of
dust
-May be organic or inorganic material
(fibrogenic or non-fibrogenic)
-Association with chronic inflammatory
response, cytokine production, and fibrosis =
restrictive lung disease

Question 4

earliest common manifestation

Answer 4

alveolitis

Question 5

honey comb lung

Answer 5

end stage fibrotic lung includes cystic space line by fibrous connective tissue

Question 6

end stage development of

Answer 6

pulmonary HTN
cor pulmonale
right heart failure
-pressure on the capillaries, all secondary impairment of capillary flow

Question 7

restrictive disease see a decrease in

Answer 7

gas exchange

Question 8

reduction in

Answer 8

oxygen diffuse capacity

Question 9

location of the lesions is directly related to

Answer 9

particle size

Question 10

1-2 microns

Answer 10

maximum penetration to alveoli

Question 11

5-10 microns

Answer 11

deposited in upper airways

Question 12

> 10 microns

Answer 12

not suspended in air

Question 13

<1 micron

Answer 13

remains suspended in air and is exhaled

Question 14

pneumoconiosis and restrictive lung disease over view

Answer 14

chronic inflammation from inhaled particles-> chronic production of inflammatory cytokines-> fibrosis of terminal airways and alveoli (honey-comb lung)-> eventual decrease gas exchange-> pulmonary HTN and cor pulmonale

Question 15

carbon partciles

Answer 15

anthracosis

Question 16

simple coal workers pneumoconiosis

Answer 16

pigmented macuoles (particularly in lymph nodes) with littl collagen

• prominent around respiratory bronchioles in upper lobes
• adjacent emphysematous changes
• usually asymptomatic

Question 17

progressive massive fibrosis

Answer 17

-pigmented fibrotic nodules with central necrosis
-interstitial fibrosis
-associated with dyspnea, black sputum, cor
pulmonale (pulmonary hypertension with right
heart failure from ventricular hypertrophy)

Question 18

co-existence of pneumoconiosis with rheumatoid

arthritis

Answer 18

Caplan’s syndrome

Question 19

silicosis

Answer 19

inhalation of silicon dioxide as quartz

• most dangerous particles between 1-5 microns: so can get into lower alveoli and respiratory bronchioles
• crystalline forms more fibrogenic
• causes both physical and chemical injury
• damages macrophage membranes

Question 20

acute silicosis

Answer 20

Unusual
 High exposure in short time
 Alveoli filled with lipoproteinaceous fluid
 Acute cor pulmonale

Question 21

chronic silicosis: simple nodular

Answer 21

multiple nodules 1-2 mm
“snowstorm” appearance on X-ray
birefringent particles in polarized light
common in upper lobes

Question 22

chronic silicosis: progressive massive fibrosis

Answer 22

conglomerate masses, >3 cm

severe symptoms

Question 23

silico TB

Answer 23

increased susceptibility: because alveolar macrophages damaged
poor granulomatous response

Question 24

can you see caplans syndrome with silicosis?

Answer 24

yes

treat with steroids but exclude TB first

Question 25

Silicosis Pathology

Answer 25

• Tiny, barely palpable, discrete nodules in upper lobes
• Nodules coalesce into hard collagenous scars
• Central softening and cavitation may lead to honeycomb lesions; also eggshell calcifications

Question 26

asbestos

Answer 26

serpitine or amphibole

Question 27

serpitine asbestos

Answer 27

soft and curly

-get trapped in upper airways

Question 28

amphibole asbestos

Answer 28

hard and brittle

- travel straight down and enter alveoli, penetrate epithelium and reside in interstitium

Question 29

what does cigarette smoking do to asbestos?

Answer 29

coats them with additional carcinogens

Question 30

non-malignant asbestos forms of diseases

Answer 30

• parietal pleural plaques
• benign effusions
• diffuse pleural fibrosis
• diffuse pulmonary fibrosis (asbestosis)
• all caused by inflammatory cytokines
• mainly in lower lobes of lungs
• get pleural plaques, made of just collagen

Question 31

malignant asbestos diseases

Answer 31

• (most common) bronchogenic carcinoma: goes along with diffuse pulmonary fibrosis
• mesothelioma: goes along diffuse pleural fibrosis

Question 32

parietal pleural plaques

Answer 32

-Proliferation of pleura with thickening and
fibrosis
Parietal pleural plaques – 10-20 years after
exposure:
-white smooth/nodular (sugar frosting on cake): all made of collagen
-acellular hyalinized collagen with slit-like spaces
-no asbestos fibers in the plaques
-asymptomatic
-not a predictor of asbestosis (diffuse disease)

Question 33

diffuse pleural fibrosis

Answer 33

usually accompanies parenchymal asbestosis

Question 34

asbestosis

Answer 34

diffuse pulmonary fibrosis (not nodular)
-lower lobes affected
-asbestosis bodies – not specific, can be seen in sputum
and lavage
-progression to progressive massive fibrosis

Question 35

is asbestos in pleural plaques?

Answer 35

NO

Question 36

how so silicosis and asbestos differ mainly?

Answer 36

asbestos in LOWER LOBES

silicosis in UPPER LOBES

Question 37

bronchogenic carcinoma

Answer 37

-risk increased by smoking
-is initiator and promoter
MOST COMMON CANCER ASSOCIATED WITH ASBESTOS

Question 38

malignant mesothelioma

Answer 38

NOT increased with smoking
-recurrent effusions
biphasic pattern

Question 39

Berylliosis: acute

Answer 39

-rare
allergic pneumonitis
rhinitis and tracheobronchitis

Question 40

Berylliosis: chronic

Answer 40

-frequent
resembles sarcoid granules
-delayed type HS (type IV)
-2 FOLD INCREASE IN LUNG CANCER

Question 41

what causes the fibrotic reaction

Answer 41

cytokine production