Restrictive Lung Disease Flashcards

1
Q

What is the definition of restrictive physiology in regards to lung volumes?

A

Lung volumes are reduced

decreased TLC, FRC, FEV1 and FVC

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2
Q

What type of work is affected by obstructive disease and during which phase of respiration?

A

increase in resistive work in both inspiration and expiration

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3
Q

What type of work does restrictive lung dz affect and during which phase?

A

increase in elastic work and affects inhalation ONLY

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4
Q

what is the composition of work of breathing?

A

elastic and resistive work

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5
Q

What does a decrease in compliance cause in elastic work/

A

increase in elastic work

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6
Q

What 3 factors affect lung compliance?

A
  1. tissue content of the lung
  2. lung H20
  3. surface tension
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7
Q

What happens to lung compliance with increased lung h20 and decreased surfactant?

A

decreased lung compliance

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8
Q

What 2 features distinguish restrictive lung dz from restrictive physiology?

A
  1. Slope of the PV curve is reduced in restrictive dz

2. DLCO/VA is decreased in restrictive dz

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9
Q

what is the difference btw restrictive lung dz and restrictive physiology?

A

restrictive lung dz is if you have a dz in your lung whereas restrictive physiology is if you are fat or have a neuromuscluar problem

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10
Q

True or False: Chest wall disease changes the slope of the PV curve

A

False, PV curve has the same slope as normal

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11
Q

T/F Restrictive chest wall disease causes gas exchange problems

A

False: gas exchange unit is fine its just the chest wall that is screwed up

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12
Q

What is the first abnormality seen in patients with pulmonary fibrosis?

A

reduced DLCO

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13
Q

What 2 criteria must be made to have mixed obstructive and restrictive dz?

A
  1. reduced lung volumes

2. FEV1/FEV

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14
Q

what is the most common cause of interstitial lung disease?

A

autoimmune

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15
Q

what is the most important factor for figuring out the type of interstitial lung disease?

A

underlying etiology (history)

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16
Q

What 2 things must you combine to make an accurate diagnosis of ILD?

A
  1. etiology

2. pathology

17
Q

what are common findings for physical exam of ILD?

A

auscultation: end-inspiratory “velcro like” crackles
distal clubbing in advanced cases
joint an skin can elude to connective tissue disease

18
Q

What is the pathology for idiopathic pulmonary fibrosis?

A

areas of normal tissue next to areas of fibrotic tissue. Heterogenous involvement and different stages of scarring and honeycombing

19
Q

what does an X-ray look like for IPF?

A

peripheral and basilar predominate reticulation, honeycombing, and ground glass

20
Q

what is an alveolar wall finding that is distinctive feature of Non-specific interstitial pneumonia (NSIP)?

A

alveolar wall thickening

21
Q

what is a radiographic finding of NSIP?

A

Diffuse fibrosis

22
Q

what is organizing pneumonia?

A

non-infectious pneumonia

23
Q

what is the treatment for organizing pneumonia?

A

steroids but they are required for 6-12 months

24
Q

what is the pathologic findings of organizing pneumonia?

A

plugs of granulation tissue and fibrosis distal to bronchioles

25
Q

how do you diagnose acute eosinophilic pneumonia?

A

> 20% eosinophils

26
Q

how do you treat eosinophilic pneumonia?

A

steroids

27
Q

what’s the radiographic finding of chronic eosinophilic pneumonia?

A

photographic negative of pulmonary edema

28
Q

what patient population does lymphangioleiomyomatosis (LAM)?

A

cystic lung disease that only affects young women

29
Q

what mutation causes LAM?

A

mutation in tuberous sclerosis gene that causes peribronchovascular proliferation of smooth muscle

30
Q

what is the common radiographic finding on LAM?

A

cysts that are distributed profusely

31
Q

how do you treat LAM?

A

mTOR inhibitors

32
Q

what is a common site of cancer in LAM?

A

angiomyolipomas (renal tumor)

33
Q

what is the characteristic pathology of sarcoidosis?

A

non-caveating granulomas

34
Q

What type of pattern of disease do sarcoid patients show/

A

restrictive, obstructive, or mixed PFT

35
Q

What is the treatment for sarcoidosis?

A

no tx necessary however can use steroids or cytotoxic drugs

36
Q

what is fibrous plaques consistent with?

A

usual interstitial pneumonia