Restrictive Lung Disease

Question 1

what is the interstitium of the lung?

Answer 1

this is the connective tissue space around the airways and vessels and the space between the basement membranes of the alveolar walls

Question 2

what are the affects of fibrosis after inflammation in the lung?

Answer 2

this reduces strechability and reduces the lung volume and compliance.

Question 3

what are the effects of restrictive lung disease on the FEV1 and FVC values?

Answer 3

this reduces both of these values but the ration is the same

Question 4

what is the presentation of restrictive lung disease?

Answer 4

> dyspnoea
shortness of breath on exertion that develops into shortness of breath at rest
heart failure
many patients may be asymptomatic

Question 5

describe CXR of a patient with interstitial lung disease

Answer 5

white fibrous tissue can be seen and the lung volume is greatly reduced

Question 6

name an acute response that leads to restrictive lung disease

Answer 6

diffuse alveolar damage

Question 7

what is diffuse alveolar damage associated with?

Answer 7

> major trauma
> chemical injury
> circulatory shock
> drugs
> infection
> autoimmune disease
> radiation

Question 8

describe the evolution of diffuse alveolar damage.

Answer 8

in the first three days of injury there is oedema which then forms a hyaline membrane. this is the exudate stage. at day 7 it enters the proliferative stage where there is interstitial inflammation and fibrosis.

Question 9

describe the histological features of DADs

Answer 9

there is protein rich oedema, fibrin, hyaline membranes and a denuded basement membrane. the walls are damaged and the air spaces filled with interstitial fluid.
there is epithelial proliferation and fibroblast proliferation trying to repair the lung.

Question 10

define sarcoidosis

Answer 10

this is a multi system granulomatous disorder of unknown aetiology. it is the commonest interstitial disease.

Question 11

what cell types would you expect to find in sarcoidosis?

Answer 11

epilethiod and giant cell granulomas

Question 12

in sarcoidosis how are the alveoli around the granulomas affected?

Answer 12

they are virtually normal

Question 13

a young man presents with acute arthralgia, erythema nodosum and a bilateral hilar lymphadenopathy. (sarcoidosis) what is the treatment and the prognosis?

Answer 13

sarcoidosis is treated with corticosteroids and this will probably resolve in 2 years

Question 14

what is the prognosis of a sarcoidosis presenting incidentally in an abnormal CXR with no symptoms?

Answer 14

it may resolve, persist or progress

Question 15

a patient with shortness of breath, a cough and an abnormal CXR has sarcoidosis. what is the prognosis?

Answer 15

it may resolve, persist or progress

Question 16

what causes hypersensitivity pneumonitis?

Answer 16

the inhalation of an organic molecular macromolecule or microorganism to which the host has become sensitised

Question 17

name some examples of hypersensitivity pneumonitis

Answer 17

pigeon fanciers lung
aspergillum
thermophilic actinomycetes

Question 18

what is the acute presentation of hypersensitivity pneumonia?

Answer 18

> fever, dry cough
chills 4-9 hours after the exposure
crackles, tachypnoea, wheeze
precipitating antibody

Question 19

what is the chronic presentation of hypersensitivity pneumonia?

Answer 19

> malaise, shortness of breath, cough
crackles, wheeze
insidious

Question 20

what type of hypersensitivity reactions are responsible for hypersensitivity pneumonitis?

Answer 20

type 3 and type 4

Question 21

what sort of cells are found in hypersensitivity pneumonitis?

Answer 21

epitheliod granulomata and foamy histiocytes

Question 22

what can cause interstitial pneumonitis?

Answer 22

> drug reaction
post infection
industrial exposure such as asbestos

Question 23

what tissues proliferate in interstitial pneumonitis?

Answer 23

smooth muscle, vascular and fibroblasts

Question 24

what are the clinical signs of interstitial pneumonitis?

Answer 24

> dyspnoea
> cough
> basal crackles
> cyanosis
> clubbing

Question 25

in what patients does interstitial pneumonitis usually develop?

Answer 25

men who are older than 50

Question 26

what is the prognosis of interstitial pneumonitis?

Answer 26

poor, it is pregressive and can go on to develop cor pulmonale, most are dead within 5 years.

Question 27

what is type 1 respiratory failure?

Answer 27

a PaO2 < 8kPa

Question 28

what is type 2 respiratory failure?

Answer 28

PaCO2 > 6.5kPa

Question 29

what is the physiological definition for restrictive lung disease?

Answer 29

a forced vital capacity < 80% of the predicted normal, this depends on size, age, gender and ethnicity.

Question 30

what are the lung causes for restriction?

Answer 30

> idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

Question 31

that are the pleural causes of restriction?

Answer 31

> pleural effusion
pneumothorax
pleural thickening

Question 32

name some skeletal causes for restriction?

Answer 32

> kyphoscoliosis
> ankylosing spondylitis
> osteoporosis
> thoracoplasty
>fractured ribs

Question 33

what are some muscular causes for restriction?

Answer 33

amyotrophic lateral sclerosis

Question 34

what are some sub-diaphragmatic causes for restriction?

Answer 34

> pregnancy

> obesity

Question 35

what is the histological hallmark of sarcoidosis?

Answer 35

non caseating granuloma

Question 36

what tests would you carry out for sarcoidosis?

Answer 36

> CXR
> bloods
> urinalysis
> ECG
> TB skin test
> eye exam
> bronchoscopy

Question 37

when would systemic steroids be given to a patient?

Answer 37

if they had cardiac, neurological or eye disease that was not responding to topical steroids

Question 38

what is the presentation for idiopathic pulmonary fibrosis?

Answer 38

> chronic breathlessness
> cough
> clubbing
> crackles
> 60-70 old men

Question 39

in idiopathic pulmonary fibrosis what treatment can be given?

Answer 39

an oral anti-fibrotic or palliative care

surgery if certain parameters are met