26. Restrictive lung disease Flashcards Preview

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Flashcards in 26. Restrictive lung disease Deck (42):
1

Volumes

1. decreased FVC
2. Decreased TLC

2

Restricted lung disease - pulmonary function test

FEV1/ FVC >= 80%

3

Types

1. poor breathing mechanics
2. interstitial lung diseases

4

Poor breathing mechanics properties

1. extrapulmonatu
2. peripheral hypoventilation
3. normal A-a gradient

5

Interstitial lung diseases properties

decreased pulmonary diffusing capacity
2. Increased A-a gradient

6

POor breathing mechanics - situations and causes

1. poor muscular effort (polio, myasthenia gravis)
2. poor structural apparatus ( scoliosis, morbid obesity)

7

causes of poor muscular effort

polio, myasthenia gravis)

8

causes of poor structural apparatus

scoliosis, morbid obesity)

9

Largerhans cell histiocytosis - also called

eosinophilic granuloma

10

Restricted lung lung disease - drug toxicity

1. bleomycin
2. busulfan
3. amiodarone
4. methotrexate

11

idiopathic pulmonary fibrosis - pathophysiology

repeated cycles of lung injury and wound healing with increased collagen deposition

12

sarcoidosis imafes and histology

1. bilateral hilar lymphadenopathy
2. nonvaseating granuloma in multiple organs

13

sarcoidosis - '' paraneoplastic

1. elevated ACE
2. hypercalcemia

14

sarcoidosis - pathophysiology og hypercalcemia

1-α hydroxylase activity of epitheliod histiocytes converts vitamin D to its active form

15

Causes of interstial lung capacity

1. ARDS
2.NRDS
3. Pneumoconioses
4. Sarcoidosis
5. Idiopathic pulmonary fibrosis
6. Goodpasture
7. Wegener
8. Eosinophilic granuloma
9. Hypersensitive pneumonitis
10. drugs

16

Idiopathic pulmonary fibrosis - mechanism

repeated cycles of lung injury and wound healing with increased collagen deposition

17

Idiopathic pulmonary fibrosis - histology

honeycomb

18

Idiopathic pulmonary fibrosis - special characteristic in clinical presentation

digital clubbing

19

normal FEV1/FVC

80%

20

obstructive FEV1/FVC

less than 80%

21

restrictive FEV1/FVC

more than 80%

22

obstructive lung volumes

increased TLC FRC RV

23

• What two lung volumes are typically decreased in patients with restrictive lung disease?

Functional vital capacity and total lung capacity

24

• Patients with restrictive lung disease typically have a FEV1/FVC ratio within what range?

≥80%

25

• What are the two general types of restrictive lung disease?


Poor breathing mechanics and interstitial lung diseases

26

• Extrapulmonary causes of restrictive lung disease are generally the result of what?

Poor breathing mechanics from muscular (e.g., polio, myasthenia gravis) or structural (e.g., scoliosis, morbid obesity) dysfunction

27

• Mechanical restrictive lung diseases have a(n) ___ A-a gradient. Interstitial lung diseases have a(n) ___ A-a gradient.

Normal; increased

28

• What infectious disease can cause poor muscular effort and lead to extrapulmonary restrictive lung disease and peripheral hypoventilation?



Polio

29

• What disease of the neuromuscular junction can cause poor muscular effort and thereby lead to extrapulmonary restrictive lung disease?

Myasthenia gravis

30

• A 32-y/o woman with neuromuscular disease, ptosis, diplopia, and muscle weakness at the day's end may have what pulmonary manifestation?

Restrictive lung disease from poor muscular effort (the patient likely has myasthenia gravis)

31

• Other than scoliosis and muscle diseases, what condition can lead to extrapulmonary restrictive lung disease?



Morbid obesity

32

• Pulmonary causes of restrictive lung disease are generally the result of what category of diseases?

Interstitial lung diseases

33

• Restrictive lung disease due to acute respiratory distress syndrome (ARDS) would have ____ (increased/decreased) diffusing capacity.

Decreased

34

• A premature infant is diagnosed with hyaline membrane lung disease. What type of restrictive is this?

Interstitial type (this is neonatal respiratory distress syndrome [NRDS], also known as hyaline membrane disease)

35

What category of interstitial lung diseases has a clear association with an environmental exposure?

Pneumoconioses (e.g., anthracosis, silicosis, asbestosis)

36

• A patient has bilateral hilar lymphadenopathy, noncaseating granulomas, and hypercalcemia. What interstitial lung disease does she have?

Sarcoidosis

37

• A patient has an autoimmune disease with hemoptysis and hematuria with renal failure. What kind of lung disease does this patient have?


Restrictive lung disease (Goodpasture syndrome)

38

• What granulomatous small-to-medium vessel vasculitis causes both glomerulonephritis and pulmonary dysfunction?

Granulomatosis with polyangiitis (Wegener)

39

• What disease can cause restrictive lung disease due to eosinophilic granulomas in the lungs?

Langerhans cell histiocytosis

40

• A patient has a cough and dyspnea at work that resolves when he leaves. What restrictive pulmonary process is likely causing his symptoms?

Hypersensitivity pneumonitis

41

• Name four drugs that can cause interstitial lung disease.

Bleomycin, busulfan, methotrexate, and amiodarone

42

• A patient has progressive dyspnea. Serum ACE and Ca2+ levels are elevated. What will a CXR show?



Bilateral hilar lymphadenopathy (this is likely sarcoidosis)