Restrictive Diseases

Question 1

restrictive diseases have reduced lung volumes because of –

Answer 1

an alteration in lung parenchyma

Question 2

– and defects in chest wall or neuromuscular apparatus may also cause respiratory restrictions

Answer 2

pleural diseases

Question 3

risk factors associated with poorer outcomes

Answer 3

older age, male, h/o cigarette smoking

Question 4

collagen vascular diseases

Answer 4

scleroderma, systemic lupus erythematosus (SLE), ankylosing spondylitis

Question 5

environmental exposures

Answer 5

medications, dust, ionizing radiation

Question 6

Idiopathic fibrotic disorders –

Answer 6

idiopathic pulmonary fibrosis (IPF)

Question 7

Infiltrative diseases -

Answer 7

sarcoidosis

Question 8

etiology of scleroderma

Answer 8

unknown

Question 9

scleroderma involves – sclerosis

Answer 9

progressive systemic

Question 10

scleroderma is characterized by progressive interstitial fibrosis with atrophy and sclerosis of many organ systems such as

Answer 10

skin, internal organs, blood vessel walls

Question 11

sclerodermic skin

Answer 11

leathery

Question 12

pulmonary scleroderma: –, sclerosis and progressive volume loss

Answer 12

diffuse interstitial infiltrates

Question 13

CXR of pulmonary scleroderma

Answer 13

fibrocystic spaces (honeycomb) mostly at lung base

Question 14

systemic lupus erythematosus is a disorder of –

Answer 14

immune regulation

Question 15

SLE: tissues are damaged by –

Answer 15

autoantibodies and immune complexes

Question 16

SLE: antibodies are directed to –

Answer 16

blood, endothelial and neuronal cells

Question 17

T/F: SLE is a significant cause of renal failure

Answer 17

true

Question 18

cardiac manifestations of SLE

Answer 18

pericarditis and endocarditis

Question 19

SLE may involve –, pneumonitis, interstitial lung diseases

Answer 19

pleural effusion

Question 20

ankylosing spondylitis is a type of – of the axial skeleton

Answer 20

spondyloarthropathy

Question 21

ankylosing spondylitis is associated with –

Answer 21

human leukocyte antigen (HLA) B27

Question 22

hallmark of ankylosing spondylitis

Answer 22

new bone formation and joint ankyloses –> rigid or bamboo spine

Question 23

ankylosing spondylitis usually involves – sites

Answer 23

extra-articular

Question 24

disease at extra-articular sites include –, aortitis, aortic insufficiency, conjunctivitis and uveitis

Answer 24

pulmonary fibrosis

Question 25

swelling and irritation of the uvea

Answer 25

uveitis

Question 26

depending on the length of exposure, pulmonary inflammation and/or fibrosis leads to acute symptoms

Answer 26

cough

Question 27

depending on the length of exposure, pulmonary inflammation and/or fibrosis leads to chronic disease

Answer 27

dyspnea and weight loss

Question 28

pulmonary disease with certain medications

Answer 28

gold, Dilantin, bleomycin, methotrexate

Question 29

seen in sandblasters, stone cutters & concrete workers who are exposed to silica dust

Answer 29

silicosis

Question 30

– seen in mushrooms growers or farmers exposed to compost & peat moss or in factory workers exposed to plastics, resins or paints

Answer 30

hypersensitivity pneumonitis

Question 31

seen in coal miners

Answer 31

coal miner's pneumoconiosis

Question 32

ionizing radiation due to occupational exposure or for cancer tx may lead to --

Answer 32

lung damage (edema --> fibrosis) and radiation pneumonia

Question 33

sarcoidosis -- changes involving the lungs and many other organ systems

Answer 33

granulomatous tissue

Question 34

cause of sarcoidosis

Answer 34

unknown

Question 35

can sarcoidosis occur in salivary glands and oral mucosa?

Answer 35

yes

Question 36

higher prevalence of sarcoidosis among

Answer 36

women and African Americans

Question 37

idiopathic pulmonary fibrosis prevalence increases with --

Answer 37

age, smoking, exposure to dust, metals, organic solvents, agriculture employment

Question 38

clinical features of idiopathic pulmonary fibrosis

Answer 38

hypoxia and digital clubbing

Question 39

idiopathic pulmonary fibrosis patients may benefit from -- in early stages

Answer 39

anti-inflammatories

Question 40

advanced idiopathic pulmonary fibrosis is associated with -- and candidate for lung transplant

Answer 40

O2 dependency and poor prognosis

Question 41

symptoms and signs of idiopathic pulmonary fibrosis: progressive -- especially with exercise

Answer 41

dyspnea

Question 42

symptoms and signs of idiopathic pulmonary fibrosis: cough (esp after deep breath) but -- not likely

Answer 42

sputnum

Question 43

symptoms and signs of idiopathic pulmonary fibrosis: cyanosis is seen in --

Answer 43

late stages

Question 44

pulmonary function changes in idiopathic pulmonary fibrosis

Answer 44

decreased TV, VC, TLC

Question 45

treatment of idiopathic pulmonary fibrosis

Answer 45

treat underlying problem; corticosteroids and cytotoxic drugs

Question 46

common feature of restrictive diseases

Answer 46

interstitial pulmonary tissue involvement

Question 47

restrictive changes are associated with pulmonary manifestations such as --

Answer 47

dyspnea

Question 48

chronic restrictive diseases may lead to --

Answer 48

hypoxemia

Question 49

T/F: many restrictive disease conditions are irreversible

Answer 49

true