Restictive lung disease - sarcoidosis, idiopathic pulmonary fibrosis, diffuse alveolar damage syndrome, hypersensitivity pneumonitis

Question 1

What part of the lung is affected by restrictive lung disease

Answer 1

the interstitium - connective tissue space around airways and vessels and space between basement membranes of alveolar walls

Question 2

Characteristics typical of restrictive interstitial lung disease

Answer 2

Reduced lung compliance - stiff lungs
Low spirometry
Reduced gas transfer
Ventilation/perfusion imbalance

Question 3

Complications of restrictive lung disease

Answer 3

hypoxic cor pulmonale, heart failure, type 1 respiratory failure

Question 4

Restrictive lung disease is interstitial inflammation. What, in very general terms, causes the inflammatory response?

Answer 4

Parenchymal (interstitial) lung injury

Question 5

What is the name of the syndrome relating to an acute inflammatory response

Answer 5

Diffuse alveolar damage syndrome

Question 6

Aetiology of diffuse alveolar damage syndrome?

Answer 6

IDIOPATHIC
Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection
Auto(immune) disease
Radiation

Question 7

What happens in the exudative stage of diffuse alveolar damage syndrome

Answer 7

Oedema

Hyaline membrane formation

Question 8

What happens in the proliferative stage of diffuse alveolar damage syndrome

Answer 8

Interstitial inflammation

Interstitial fibrosis

Question 9

Histological features of DADS

Answer 9

protein rich oedema
fibrin
hyaline membranes
denuded basement membranes
epithelial proliferation
fibroblast proliferation
scarring

Question 10

Granulomatous responses are a sub type of chronic response to lung injury. What 2 diseases do you need to know for it?

Answer 10

Sarcoidosis

Hypersensitivity pneumonitis

Question 11

What is sarcoidosis?

Answer 11

A multisystem granulomatous disorder of unknown aetiology

Question 12

Is sarcoidosis caesating?

Answer 12

No (but TB is)

Question 13

Histopathology of sarcoidosis

Answer 13

epithelioid and giant cell granulomas
little lymphoid infiltrate
variable associated fibrosis

Question 14

Who does sarcoidosis normally affect?

Answer 14

Young adults

Female more than male

Question 15

Presentation of sarcoidosis

Answer 15

acute arthralgia (knee pain)
erythema nodosum (rash)
bilateral hilar lymphadenopathy
incidental abnormal CXR
dyspnoea
cough

Question 16

Prognosis of sarcoidosis

Answer 16

Most resolve

Question 17

Prognosis of DADS

Answer 17

fatal

Question 18

Investigations with sarcoidosis

Answer 18

CXR
Blood test - serum calcium and ACE
Biopsy

Question 19

Antigens of hypersensitivity pneumonitis

Answer 19

Thermophilic actinomycetes
Bird/animal proteins
Fungi
Chemicals

Question 20

Acute presentation of hypersensivity pneumonitis

Answer 20

Fever, dry cough, myalgia
Chills 4-9 hours after exposure
Crackles, tachypnoea, wheeze
Precipitating antibody

Question 21

Chronic presentation of hypersensitivity pneumonitis

Answer 21

Insidious
Malaise, SOB, cough
Low grade illness
Crackles and some wheeze

Question 22

Where in the lung is hypersensitivity pneumonitis common?

Answer 22

Upper zone

Question 23

What type of hypersensitivity reaction is in hypersensitivity pneumonitis?

Answer 23

Type 3 and 4

Question 24

Aetiology of interstitial pneumonitis (v bad one)

Answer 24

IDIOPATHIC or cryptogenic

Connective tissue diseases
Drug reaction
Post infection
Industrial exposure

Question 25

3 things making up the histopathology of interstitial pneumonitis

Answer 25

Patchy interstitial chronic inflammation Type II pneumocyte hyperplasia Smooth muscle and vascular proliferation

Question 26

Typical patient having idiopathic pulmonary fibrosis

Answer 26

elderly male progressive disease

Question 27

What do all three chronic responses go to if they do not resolve (or haven't died already)

Answer 27

Fibrosis and end stage honeycomb lung

Question 28

Definition of restriction

Answer 28

FVC <80% predicted

Question 29

What are the lung causes of restriction (3 to remember)

Answer 29

sarcoidosis idiopathic pulmonary fibrosis hypersensitivity pneumonitis

Question 30

Pleural causes for restriction

Answer 30

pleural effusion pneumothorax pleural thickening

Question 31

Skeletal causes for restriction

Answer 31

kyphoscoliosis ankylosing spondylitis thoracoplasaty rib fractures

Question 32

Muscle causes for restriction

Answer 32

amyotrophic lateral sclerosis

Question 33

sub-diaphragmatic causes for restriction

Answer 33

obesity | pregnancy

Question 34

Investigations for sarcoidosis

Answer 34

``` CXR Pumonary function test Bloods Urinalysis ECG TB skin test eye exam HR CT Surgical biopsy ```

Question 35

Treatment of sarcoidosis

Answer 35

None if mild NSAIDS Topical steroids Systemic steroids

Question 36

Treatment for idiopathic pulmonary fibrosis

Answer 36

Oral antifibrotic Palliative treatment Transplant