Restrictive Lung diseases

Question 1

what is the chief problem of all restrictive lung diseases?

Answer 1

can’t get air in, but have normal exhale (but of course exhale is small too bc the amount of air u got in in the first place in order to exhale it is small)

Question 2

how are restrictive lung diseases classified?

Answer 2

reduced lung volumes with normal expiratory flow rates

Question 3

what is the usual ultimate pathological condition that most ppl with RLDs end up with?

Answer 3

diffuse fibrosis

Question 4

Restrictive lung dysfunction. (RLD)

Answer 4

side effect of reduced compliance of lungs or of chest wall for a number of different reasons.

Question 5

is RLD a disease process?

Answer 5

no, its a side effect

Question 6

what are the values for RLD?

Answer 6

transpulmonary pressure is kept relatively constant due to lack of elasticity, this causes dec. of volume that can get in
inc. in RR to try to help but then that gives up and u are left with dec. RR, dec. FVC, dec. TLC, dec. VC, dec. ERV dec. IRV, dec. FRC (ERV+RV)

Question 7

what is work of breathing in RLD? what is normal?

Answer 7

greater than or equal to 25%, norm is 5%

Question 8

might these ppl need o2 during therapy?

Answer 8

yes, maybe up to 1-6L with exercise

Question 9

what might be reasons for RLD?

Answer 9

pollutants causing injury to lung–>scarring and fibrosis

injury to chest wall: paralysis, torn mm, broken rib, obesity

Question 10

classic signs of RLD?

Answer 10

Tachypenea (inc. RR)
hypoxemia
dec. breath sounds- with rales 
non productive dry, irritating cough (#1 symp. that brings ppl in to doctor) 
emaciated appearance

Question 11

what do ppl with RLD usually die from?

Answer 11

cor pulmonale

Question 12

why do ppl with RLD get cor pulmonale?

Answer 12

hypoxemia and fibrosis of capillaries leads to pulm. HTN and creates inc. pressure for the R ventricle to pump against until it gives out.

Question 13

ppl with chronic bronchitis have a _______ cough, but ppl with RLD have a ___________ cough!

Answer 13

bronchitis= productive! 
RLD= non productive

Question 14

PULMONARY FIBROSIS- types

Answer 14

sarcoidosis
lupus
IPF
scleroderma
rheumatic disease

Question 15

sarcoidosis

Answer 15

granulomatous disease of unknown etiology

Question 16

what is it that causes granulomas at the site injection if u have sarcoidosis?

Answer 16

kviem antigen

Question 17

what does fibrosis alveolitis look like on an X ray?

Answer 17

honeycomb, scarring

Question 18

S&S of sarcoidosis

Answer 18

dry, irritating cough, general weakness, fatigue, weight loss, malaise, fever

Question 19

tx for sarcoidosis

Answer 19

corticosteroids and immunosuppressive drugs

Question 20

Lupus

Answer 20

systemic autoimmune disease with varied clinical manifestations

Question 21

who does lupus affect most often?

Answer 21

women ages 15-45

Question 22

what lung sounds do ppl with lupus have?

Answer 22

dec. souds, friction rub, rales,

Question 23

what do ppl with lupus present like?

Answer 23

butterfly rash, cyanotic, may have raynauds

Question 24

rheumatoid disease

Answer 24

dec. chest wall compliance secondary to systemic and pulmonary rheumatoid disease.

Question 25

who is affected by rheumatoid disease?

Answer 25

females more than males, 50-60 years old

Question 26

Scleroderma

Answer 26

an autoimmune disease that affects the endothelial cells of blood vessels and connective tissues causing fibrosis in organs. non-reversible damage.

Question 27

who is affected by scleroderma?

Answer 27

F >M x5

Question 28

Idiopathic pulmonary fibrosis (IPF)

Answer 28

chronic lung disease that causes fibrosis of alveoli and capillary network, differentiated only by lack of any causative factor or environmental agent.

Question 29

chief sign of IPF

Answer 29

slow, progressive dyspnea

Question 30

Who gets IPF?

Answer 30

Middle aged men>women

Question 31

life expectancy of someone diagnosed with IPF?

Answer 31

50% live 5 years, R sided heart failure, or lack of O2

Question 32

values for IPF

Answer 32

dec. TLC, VC, FRC (ERV+RV), inc. venilation-perfusion mismatch

Question 33

when in doubt, how do u treat all restrictive lung diseases?

Answer 33

immunosuppressors and corticosteroids

Question 34

4 categories of restrictive environmental lung diseases

Answer 34

infectious agents organic dusts inorganic dusts gases

Question 35

pneumoconioses

Answer 35

inhalation of inorganic dust- COAL WORKERS

Question 36

silicosis

Answer 36

inhaling silicas (crystalline silicone dioxide)- mining, grinding, polishing rock, ship building,

Question 37

asbestos

Answer 37

inhaling silicates- sheet metal, ships, asbestos mines

Question 38

what sound do u here both with asbestos and coal workers pneumonia?

Answer 38

rales

Question 39

irritant gasses that are inhaled

Answer 39

``` ammonia sulfur dioxide chlorine nitrogen dioxide ozone phosgene ```

Question 40

what happens when irritant gasses are inhaled?

Answer 40

epithelial injury, hyperemia, edema, mucosal sloughing, coughing, dyspnea, cyanosis-pulmonary fibrosis from significant exposure

Question 41

restrictive lung diseases caused by spine and chest wall pathology

Answer 41

``` kyphosis scoliosis kyphoscoleosis (lateral and posterior curvature) lordosis pectus excavatum- scoop chest pectus carinatum- pigeon chest ```

Question 42

S&S of restrictive diseases caused by spine and chest wall

Answer 42

dyspnea, frequent pneumonia, respiratory insufficiency, hypoxia, hypercapnia, risk of cardiac failure

Question 43

restrictive lung diseases caused by the neuromuscular system

Answer 43

myasthenia gravis guillan barre ALS quadriplegia

Question 44

myasthenia gravis

Answer 44

autoimmune disease, body destroys its own ACH receptors= not enough ACH= weak mm, including resp. mm treat with anticholinesterase drugs and steriods

Question 45

Guillan Barre syndrome

Answer 45

acute inflammatory polyneuritis, idiopathic demyelination of peripheral nerves, ascending paralysis, need to get to a hospital before it paralyzes your diaphragm, after that scary time (1-3 weeks) (as long as u don't get pneumonia) you should start to heal up in 2-4 weeks.

Question 46

ALS

Answer 46

inflammatory or neoplastic disease of the spinal cord

Question 47

quadriplegia- what level of injury affects the diaphragm?

Answer 47

C3,C4,C5

Question 48

what level of injury affects the scalenes?

Answer 48

C5-6

Question 49

what level affects the intercostals?

Answer 49

T1-T11

Question 50

what level effects the abdominals

Answer 50

T8-T12

Question 51

what is defined as an acute inflammatory process that affects the gas exchange units of the lungs?

Answer 51

pneumonia!

Question 52

in pneumonia, what happens in response to inflammation?

Answer 52

fluid and RBCs pour into the alveoli, phagocytes also pour into the infected area to engulf and kill bacteria, then the alveoli undergo "consolidation" and become filled with fulid, looking milky.

Question 53

what happens if the adjacent lining of the pleural cavity gets involved in pneumonia?

Answer 53

pleural effusion

Question 54

S&S of pneumonia

Answer 54

malaise, chills, fever, cough, chest pain, dyspnea