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Pulmonary > Restrictive Lung Diseases (Zaman) > Flashcards

Restrictive Lung Diseases (Zaman) Flashcards

1
Q

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

A 
vascular (PAH)
obstructed airway (asthma, COPD)
Interstitial disease (fibrosis)
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2
Q

What is the definition of restrictive lung disease?

A

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

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3
Q

What are the classification of restrictinve lung disease

A

intrinsic/parenchymal= ILD or resection of lung tissue

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

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4
Q

Pleural Effusion is (intrinsic or extrinsic) lung disease?

A

extrinsic

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5
Q

What does the “meniscus sign” indicate on CXR?

A

pleural effusion

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6
Q

What is the lung interstitium?

A

the extra-vascular and extra-pleural space

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7
Q

What parts of the interstitium does ILD involve?

A

CT and alveolar wall

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8
Q

T or F: ISD can be infectious or caseous

A

False: neither but it is due to an inflammatory process

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9
Q

What is the consequence of the inflammation on ILD?

A

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

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10
Q

Describe the structure of normal lung interstitium.

A
  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
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11
Q

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

A

type II epithelial cells

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12
Q

Describe the structure of lung interstitium in ILD

A

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

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13
Q

Describe the pathophysiology of ILD

A

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

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14
Q

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

A

increased RR

decrease size of breaths = more shallow

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15
Q

How are the etiologies of ILD subdivided?

A

known and unknown and granulomatous and non-granulomatous

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16
Q

WHat is the likely etiology of pneumonia in a farmer?

A

hypersensitivity pneumonitis

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17
Q

Drug induced ILD is (granulomatous or non-granulomatous)

A

non-granulomatous

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18
Q

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

A

non-granulomatous

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19
Q

What are examples of drugs known to cause ILD

A

bleomysin, amiroderone, nitrofurantion

20
Q

What inorganic dusts are granulomatous etiologies of ILD?

non-granulomatous?

A

granulomatous: berylliosis

non-granulomatous: asbestos, coal, silica

21
Q

Sarcoidosis is (granulomatous or non-granulomatous)

A

granulomatous

22
Q

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

A

idiopathic interstitial pneumonias

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

23
Q 
The hypoxemia of ILD is due to?
A. Hypoventilation
B. VA/QC mismatch 
C. Diffusion defect
D. Right to left shunt
A

B. VA/QC mismatch

V = 0

24
Q

pts with ILD will be (hypo or hyper capnic)

A

hypocapnic = inc RR to compensate for dec lung volume

25
Q

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

A

widened

26
Q

ILD pts have exercised induced ____

A

hypoxia

sorry, didn;t know how to ask this

27
Q 
Will pts wth ILD have..
Reduced expiatory flow?
Reduced TLC?
Reduced VC?
Reduced diffusing capacity?
A

No
Yes
Yes
Yes

28
Q

What is the most common symptom of ILD?

A

dyspnea

29
Q

What are common signs of ILD?

A

Clubbing of digits
Tachypnea
Crackles/”velcro” rales

Late DZ: cor-pulomanale, cyanosis

30
Q

Why is cor pulomonale a consequence of late ILD?

A

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

31
Q

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

A

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

32
Q

How is the diagnosis of ILD made?

A

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

33
Q

What is BAL?

A

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

34
Q

What are the major inflammatory cells in alveoli?

A

macrophages (90%)

35
Q

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

A

sarcoidosis

36
Q

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

A

lung resection (Pneumonectomy)

37
Q

Name disorders of the pleura that cause ILD?

A

mesothelioma, pleural effusion, pheumothorax

38
Q

Name disorders of the chest wall that cause ILD?

A

Kyphoscoliosis

39
Q

Pathophysiology of hypoxemia in ILD is due mainly to …

A

V/Q mismatch.

40
Q

Describe the clinical features of ILD

A

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

41
Q

Compliance is (inc or dec) in ILD

A

decreased

42
Q

What ILD are fibrosis?

A

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

43
Q

What ILD are granulomatous?

A

sarcoidosis

hypersensitivity pneumonia

44
Q

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

A

Loeffler syndrome
Drug allergy related
Idiopathic chronic eosinophillic pneumonia

45
Q

What ILDs are smoking related?

A

Desquamative interstitial pneumonia

Respiratory bronchiolitis

46
Q

Idiopathic pulmonary fibrosis is (uni or bi lateral)

epidemiology?

A

bilateral
Males > Females
60+

47
Q

What is pneumoconioses?

A

non-neoplastc lung reaction to inhation of mineral dust

Pulmonary (31 decks)

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