Restrictive Lung Diseases (Zaman) Flashcards Preview

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Flashcards in Restrictive Lung Diseases (Zaman) Deck (47):
1

What type of lung DX has normal lung volume?
Dec lung volume?
Inc lung volume?

vascular (PAH)
obstructed airway (asthma, COPD)
Interstitial disease (fibrosis)

2

What is the definition of restrictive lung disease?

disease in which the expansion of the lung is limited WITHOUT airway obstruction

3

What are the classification of restrictinve lung disease

intrinsic/parenchymal= ILD or resection of lung tissue

extrinsic/extra-parenchymal= disorders of pleura, chest wall, or neuromuscular disorders

4

Pleural Effusion is (intrinsic or extrinsic) lung disease?

extrinsic

5

What does the "meniscus sign" indicate on CXR?

pleural effusion

6

What is the lung interstitium?

the extra-vascular and extra-pleural space

7

What parts of the interstitium does ILD involve?

CT and alveolar wall

8

T or F: ISD can be infectious or caseous

False: neither but it is due to an inflammatory process

9

What is the consequence of the inflammation on ILD?

excess growth of CT --> distortion of lung parenchyma --> dysfunction of gas exchange

10

Describe the structure of normal lung interstitium.

1. parenchymal cells = type I and II alveolar epithlial cells, endothelial cells
2. Inflammatory cells = macrophages > lymphocytes > neutrophils
3. CT elements = fibroblasts, collagen, elastin, proteoglycans

11

What repopulates type I alveolar epithelial cells when they are damaged?

type II epithelial cells

12

Describe the structure of lung interstitium in ILD

loss of type I cells, compressed pulm capilaries, increased CT --> thickened alveolar wall

13

Describe the pathophysiology of ILD

Injury to the alveolar epithelial layer leads to tissue damage and cell death.

--> damage to the basement membrane and the release of a variety of factors: TGF-B, IGF-1, LGF, CTGF, PDGF

--> stimulate fibroblasts to produce extracellular matrix components, particularly collagen

-->fibrous-rich regions impedes the recovery of the normal epithelial cell layer

14

How do pts compensate for dec long volume assc with ILD?

increased RR
decrease size of breaths = more shallow

15

How are the etiologies of ILD subdivided?

known and unknown and granulomatous and non-granulomatous

16

WHat is the likely etiology of pneumonia in a farmer?

hypersensitivity pneumonitis

17

Drug induced ILD is (granulomatous or non-granulomatous)

non-granulomatous

18

External radiation induced ILD is (granulomatous or non-granulomatous)

non-granulomatous

19

What are examples of drugs known to cause ILD

bleomysin, amiroderone, nitrofurantion

20

What inorganic dusts are granulomatous etiologies of ILD?
non-granulomatous?

granulomatous: berylliosis

non-granulomatous: asbestos, coal, silica

21

Sarcoidosis is (granulomatous or non-granulomatous)

granulomatous

22

What are the ILD of unknown etiologies that are non-granulomatous?

idiopathic interstitial pneumonias
ILD with connective tissue diseases (RA, lupus, scleroderma, dermatomyositis)

23

The hypoxemia of ILD is due to?
A. Hypoventilation
B. VA/QC mismatch
C. Diffusion defect
D. Right to left shunt

B. VA/QC mismatch

V = 0

24

pts with ILD will be (hypo or hyper capnic)

hypocapnic = inc RR to compensate for dec lung volume

25

ILD pts will have a normal or widened A-a gradient?

widened

26

ILD pts have exercised induced ____

hypoxia

(sorry, didn;t know how to ask this)

27

Will pts wth ILD have..
Reduced expiatory flow?
Reduced TLC?
Reduced VC?
Reduced diffusing capacity?

No
Yes
Yes
Yes

28

What is the most common symptom of ILD?

dyspnea

29

What are common signs of ILD?

Clubbing of digits
Tachypnea
Crackles/"velcro" rales

Late DZ: cor-pulomanale, cyanosis

30

Why is cor pulomonale a consequence of late ILD?

the RV has to work harder to pump blood through the compressed capillaries (due to increased intersititium)

31

What are the radiological findings assc with early, middle, and late ILD?

early: ground glass
middle: reticular, nodular, reticulo-nodular
late: honeycombing

32

How is the diagnosis of ILD made?

H&P + imaging studies for comparison + PFTs + BAL and lung biopsy

33

What is BAL?

Broncho-Alveolar Lavage: stick a bronchoscopre in and flush alveoli with saline and then suction out saline to study contents of alveoli

34

What are the major inflammatory cells in alveoli?

macrophages (90%)

35

If more lymphocytes are found than macrophages in a BAL sample what DZ might you expect?

sarcoidosis

36

How could a pt have decrease lung volume without alteration of the structure of the interstitium?

lung resection (Pneumonectomy)

37

Name disorders of the pleura that cause ILD?

mesothelioma, pleural effusion, pheumothorax

38

Name disorders of the chest wall that cause ILD?

Kyphoscoliosis

39

Pathophysiology of hypoxemia in ILD is due mainly to ...

V/Q mismatch.

40

Describe the clinical features of ILD

dyspnea, tachypnea, hypoxemia, infiltrates, restrictive changes in PFTs

41

Compliance is (inc or dec) in ILD

decreased

42

What ILD are fibrosis?

idiopathic pulmonary fibrosis
Nonspecific interstitial pneumonia
Cryptogenic organizing pneumonia
Associated with collagen vascular disease
Pneumoconiosis
Associated with therapies (drugs, radiation)

43

What ILD are granulomatous?

sarcoidosis
hypersensitivity pneumonia

44

WHat ILDs are are characterized with the infiltration and activation of eosinophils

Loeffler syndrome
Drug allergy related
Idiopathic chronic eosinophillic pneumonia

45

What ILDs are smoking related?

Desquamative interstitial pneumonia
Respiratory bronchiolitis

46

Idiopathic pulmonary fibrosis is (uni or bi lateral)
epidemiology?

bilateral
Males > Females
60+

47

What is pneumoconioses?

non-neoplastc lung reaction to inhation of mineral dust