Restrictive Lung Diseases

Question 1

In general, what happens to lung volume in restrictive diseases

Answer 1

it is reduced

Question 2

when considering restrictive diseases, what is our broad definition - location in lung and pathologic process

Answer 2

inflammatory dz of the lower respiratory tract (alveolar dz) - fibrosis

Question 3

what do we see on CXR for restrictive lung dz

Answer 3

reduction in lung volume - see the diaphragms elevated

Question 4

define intrinsic restriction and provide example

Answer 4

restriction due to alterations in lung - interstitial fibrosis

Question 5

define extrinsic restriction

Answer 5

restriction due to alterations in surrounding structure

Question 6

there are 2 types of intrinsic lung disorders causing restriction. In addition to interstitial lung dz, what is the other?

Answer 6

resection of the lung tissue

Question 7

(pathoma) - what is the PFT definition of restrictive dz (3 things)

Answer 7

1) dec FEV1
2) greater dec FVC
3) inc FEV1/FVC ratio

Question 8

for extrinsic disorders causing restriction of lung, what are the 3 main categories?

Answer 8

1) dz of pleura
2) dz of chest wall
3) neuromuscular disorders

Question 9

for interstitial lung dz, what is the predominantly affected component (1) of what structure (2)

Answer 9

1) connective tissue

2) alveolar wall

Question 10

ILD affects 2 things - lung volume and diffusion capacity. what is the most important determinant of diffusion capacity?

Answer 10

surface area

Thus in ILD, volume is dec, so surface area is dec, and diffusion capcity is dec

Question 11

what happens to expiratory airflow in ILD

Answer 11

it is not affected; this is not an obstructive disorder

Question 12

Compare the percentages of macrophage, lymphocyte, and neurophil in lung vs peripheral blood (just most common to least common)

Answer 12

blood - neutrophils, lymphocytes, then monocytes

Lung - macrophages, lymphocytes, then neutrophils least common

Question 13

comparing the two etiologies of restrictive ILD, granulomatous and nongranulomatous, which one is more lethal, i.e. more fibrotic

Answer 13

nongranulomatous is more fibrotic, and thus more lethal

granulomas are still reversible, thus less fibrotic

Question 14

if you see nongranulomatous ILD, what should be your first thought as to cause of the ILD?

Answer 14

look at medications pt may be taking

Question 15

what 3 drugs should you be worried about in pt w/ nongranulomatous ILD

Answer 15

1) amiodarone - one of most common anti-arrhythmic drugs (MUST KNOW)
2) Bleomycin
3) nitrofurantion

Question 16

what form of therapy, not a drug, can cause nongranulomatous ILD?

Answer 16

external radiation

Question 17

if you do not know the etiology of the ILD fibrosis, and you see granulomas, what is the the dx

Answer 17

sarcoidosis - it is a dx of exclusion

Question 18

If you do not know the etiology of the ILD and you do not see granulomas (nongranulomatous), what are the two broad categories of causes

Answer 18

1) Idiopathic interstitial penumonias

2) ILD w/ connective tissue diseases

Question 19

what are 4 environmental causes of ILD fibrosis?

Answer 19

1) asbestos fibers
2) coal dust
3) cotton dust
4) pigeon droppings

Question 20

the hypoxemia of ILD is mostly due to what?

Answer 20

V/Q mismatch - V is decreased compared to Q

Question 21

what is the CO2 status of a patient with ILD? and why?

Answer 21

ILD - hypocapnia due to hyperventilation

Question 22

what happens to the A-a gradient in ILD

Answer 22

A-a gradient increases

Question 23

what is the hallmark finding of ILD (regarding oxygen status)

Answer 23

Exercise induced hypoxemia

says could be on exam

Question 24

Define exercise induced hypoxemia in terms of A-a gradient

Answer 24

A-a gradient is normal at rest, but it increases during exercise

Question 25

PFT in ILD - what are hallmark findings 1) TLC 2) VC 3) diffusing capacity 4) expiratory airflow

Answer 25

1) reduced TLC 2) reduced VC 3) reduced diffusing capacity 4) normal expiratory airflow

Question 26

again, recall the definition of restrictive lung dz in terms of FEV1, FVC, and FEV1/FVC ratio

Answer 26

FEV1 is dec FVC is dec even more FEV1/FVC is inc (this might be tested - know how to diff from obstructive dz)

Question 27

To differentiate obstructive vs restrictive pathology, what values do we look for in the FEV1/FVC ratio for each (ranges)

Answer 27

obstructive - FEV1/FVC less than 70 | restrictive - FEV1/FVC greater than 80

Question 28

what is the most common symptom of ILD?

Answer 28

dyspnea

Question 29

what are the common clinical signs of ILD? 1) breathing pattern 2) auscultation of lungs 3) inspection of PVD 4) heart pathology 5) oxygen status

Answer 29

1) tachypnea 2) crackles "velcro" rales 3) digital clubbing 4) cor-pulmonale, late 5) cyanosis, late

Question 30

what is the buzzword radiologic finding of ILD that suggests end stage lung dz

Answer 30

honeycomb

Question 31

what is the buzzword radiologic finding of ILD that suggests early-stage lung dz

Answer 31

ground glass

Question 32

what is the buzzword radiologic finding of ILD that suggests in-between stage lung dz

Answer 32

interstitial markings (reticulo-nodular)

Question 33

is ground glass appearance reversible?

Answer 33

yes - can be treated

Question 34

what are the 5 components of diagnosis of ILD

Answer 34

1) extensive history 2) physical exam - evaluate symptoms and signs 3) look at radiographs 4) PFTs 5) BAL and lung biopsy (not necessary every time)

Question 35

Know about BAL

Answer 35

Muthiah told this guy to talk about it, it must be impt

Question 36

know that lung resection can cause restrictive pathology

Answer 36

remove part of lung, TLC must decrease, and so on

Question 37

in what fraction of patients can the dx of restrictive lung disease be made with just PFT and radiology? what fractioin requires lung biopsy?

Answer 37

PFT + radiology - 2/3 of pts | lung biopsy - 1/3