Lecture 8 - restrictive disease

Question 1

What is the def of restrictive lung disease?

Answer 1

Loss of compliance of the lungs and/or chest wall which prevents the lungs from expanding fully.

Question 2

what are th expected PFTs in restrictive lung disease?

VC, IC, TLC, RV, DLCO

Answer 2

↓ VC
↓ IC
↓ TLC
Normal or ↓ RV

↓ DLCO if restriction pulmonary in origin

Question 3

S/s of restrictive lung disease are what in a non-advanced state?

Answer 3

Tachypnea
Decreased breath sounds : 
dry inspiratory rales
Dyspnea
Cough: dry, irritating, non-productive

Question 4

S/s of restrictive lung disease are what in a advanced state?

Answer 4

V/Q mismatch -> hypoxemia -> pulmonary hypertension -> cor pulmonale

Weight loss: increased work of breathing, decreased appetite

Question 5

What is the name of abnormal healing response to multiple microscopic sites of acute alveolar injury that progress to fibrosis.

Answer 5

Interstitial Pulmonary Fibrosis

Question 6

Whats are the causes of inter.pulm.fibro?

Answer 6

Occupational and environmental exposure: Inorganic dusts (silica, coal), Toxic gases, Drugs (ex: amiodarone, anticoag)
Poisons

Connective tissue disorders
- i.e. RA, systemic sclerosis, lupus

Genetic link

Question 7

What is the most common cause of lung restriction?

Answer 7

Idiopathic Pulmonary Fibrosis

Question 8

What is a Idiopathic Pulmonary Fibrosis

Answer 8

Chronic progressive irreversible disease of unknown cause.

Question 9

what are possible causes of idio.pulm.fibro?

Answer 9

Risk factors: epithelial injury, genetic factors, maybe infection, microbes
Older age -> mor at risk, micro injury
Smoking, reflux (RGO) chronic, sleep apnea, ongoing reasearch

Question 10

What are the s/s of idio.pulm.fibrosis?
Resp
UE
Body structure and functions

Answer 10

Fatigue

Chronic unproductive cough

Dyspnea: on exertion initially increase at rest with disease progression

Digital clubbing

Rapid/shallow breathing

↓ chest expansion

Cyanosis as disease progresses

Weight loss, decrease in appetite

Sleep disturbances with loss of REM sleep

Question 11

what are the pulm fct of idio.pulm.fibrosis?
Lung capacities
FEV1, FVC
Compliance
DLCO
Hypoxemia

Answer 11

↓ static lung volumes and capacities
Spirometric function preserved
↓ lung compliance 
↓ DLCO
hypoxemia with exercise initially -> evident at rest or exaggerated by exercise with disease progression

Question 12

What is an Interstitial Pulmonary Fibrosis?

An it’s process?

Answer 12

Abnormal healing response to multiple microscopic sites of acute alveolar injury that progress to fibrosis.

Triggered by alveolitis -> response of lung tissus cells -> accumul of fibro & myo blasts -> collagen -> scarring

Question 13

What are the x-rays findings with pulm fibrosis?

Answer 13

Interstitial pattern is rough with evidence of honeycombing in the bases.

Question 14

Explain the restrictive /both lung disease cascade

Answer 14

Diapo 13-14 lecture 8

Question 15

what are the tx for idio.pulm.fibro (8)?

Answer 15

Antifibrotics: Nintedonib, pirfenidone
Smoking cessation
Supplemental O2
Antibiotic therapy for secondary infections
Nutritional support
Candidates for single or double lung transplantation

Might increase mortality rates:
Corticosteroids
Immunosuppressive therapy: cyclophosphamide

Question 16

what ar the 2 occupational lung diseases?

Answer 16

Mineral dusts (Pneumoconiosis) 
Asbestos, coal and silica

Organic dusts (Hypersensitivity pneumonitis)

Question 17

Silicosis:

where does it come from?

Answer 17

Silica dust: Byproduct of industry i.e. Quarry work, sandblasting, pottery making, stone masonry, mining (coal, copper, tin)

Question 18

Silicosis: when is the onset?

Answer 18

Onset 15-20 years following initial exposure.

Question 19

Silicosis: what does it cause?

Answer 19

Hard nodular deposits in lung parenchyma & peribronchial vascular regions.
- affects ULs > LLs of the lung

Question 20

Silicosis: whats the s/s

Answer 20

Asymptomatic or dry cough &

mild dyspnea

Question 21

Silicosis: whats the complication?

Answer 21

Complication: severe pulmonary
restriction -↓ lung volumes,
hypoxia, severe dyspnea,
predisposition to TB

Question 22

Coal dust:

• whats the name
• whats the onset
• what does is causes

Answer 22

coal workers pneumoconiosis

“Black lung disease” – 10-15 year latency

Small black nodules or areas of discoloration.

Question 23

T/F pneumoconiosis is a Pathogenesis related to quantity of silica (amoong others) in the dust.

Answer 23

T

Pathogenesis related to quantity of coal dust and level of quartz (silica) in the dust.

Question 24

T/F pneumoconiosis is a Chronic bronchitis

Answer 24

T

Chronic bronchitis – black secretions.

Question 25

What kind of fibrosis pneumoconiosis is?

Answer 25

progressive fibrosis

Question 26

What pneumoconiosis can form?

Answer 26

Form of centrilobular emphysema

also common.

Question 27

What causes asbestos in the lungs?

Answer 27

Pleural effusions, plaque development, asbestosis, asbestos-related malignancies

Question 28

What is the pathogenesis of asbestos?

Answer 28

Pathogenesis:
Macrophages engulf asbestos fibers in
lung and pulmonary interstitium - fibrous
tissue deposition.

Honeycomb appearance of lung parenchyma.

Pulmonary carcinoma, malignant mesothelioma.

Question 29

Asbestos causes:
A) obstruction/restruction lund disease
B) Incr/decr DLCO

Answer 29

Restrictive lung disease, ↓DLCO

Question 30

What are the finding of asbestosis in an x-ray? On a CT-scan

Answer 30

x-ray: Asbestosis with calcific pleural plaques and an interstitial parenchymal pattern.

Computed tomogram demonstrating calcific asbestosis-related pleural plaques in the right posterior region (arrows).

Question 31

What causes hypersensitivity pneumonitis (6)?

Whats the other name of disease?

Answer 31

Caused by microorganism, animal or insect protein
 moldy hay (Farmer lung)
 mushroom compost (Mushroom-worker lung)
 bird droppings (Pigeon-breeder lung)
 moldy cork ( Suberosis)
 moldy barley ( Malt-worker lung)
 insect dust ( Miller lung)

extrinsic allergic alveolitis

Question 32

hyper.pneumo causes what rxn?

Answer 32

Acute inflammatory response – lymphocytes, plasma cells & eosinophils in alveolar septa and around bronchioles

Question 33

hypr.pneumo: obstructive or restrictive?

Answer 33

Obstructive pattern acutely

Restrictive pattern with chronic exposure.

Question 34

hyper.pneumo: what does it look like on x-rays?

Answer 34

CXR similar regardless of type of organic dust inhaled - varies according to the intensity of exposure.

Early stages: reversible, multiple, small (1- to 3-mm), nodular radiodensities scattered bilaterally; lung apices may be spared

Repeated exposures: chronic interstitial (honeycomb) pattern with upper lobe predominance.

Question 35

How do we treat restrictive lung disease (5)?

Answer 35

Avoidance of exposure to causative agent

Supportive interventions: Smoking cessation
Supplemental O2
Good nutrition

Meds:
Antibiotics for secondary pulmonary infection
Corticosteroids, interferon, collagen-inhibiting agent, cytotoxic drugs (cyclophosphomide, axathioprine)

Temporary invasive or noninvasive mechanical ventilation

Lung transplantation

Question 36

What are the interventions in physio?

Answer 36

Interventions to increase ventilation:
Breathing exercises
Chest mobility, posture exercises

Pulmonary rehabilitation:

Progressive exercise training -> maximize remaining lung function and -> activity tolerance
- monitor for hypoxemia during exercise!!!

Respiratory muscle strength and endurance training

• Paced breathing/ energy conservation techniques
• Supplemental oxygen for hypoxemic patients

Question 37

where do you see the honeycomb pattern?

Answer 37

Hypersensitivity pneumonitis

Pulmonary fibrosis

Asbestosis