Restrictive lung diseases

Question 1

What are some lung causes of restrictive lung disease?

Answer 1

Interstitial lung diseases

• idiopathic pulmonary fibrosis
• sarcoidosis
• hypersensitivity pneumonitis

Question 2

What are some pleural causes of restrictive lung disease?

Answer 2

Pleural effusion
Pneumothorax
Pleural thickening

Question 3

What are some skeletal causes of restrictive lung disease?

Answer 3

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty (old TB treatment)
Rib fractures

Question 4

What is a muscular cause of restrictive lung disease?

Answer 4

Amyptrophic lateral sclerosis

Question 5

What are some sub diaphragmatic causes of restrictive lung disease?

Answer 5

Obesity

Pregnancy

Question 6

What are interstitial lung diseases?

Answer 6

Diseases that cause a thickening of the interstitial and can result in pulmonary fibrosis

Question 7

What are the main 3 types if interstitial lung disease?

Answer 7

Sarcoidosis
Idiopathic pulmonary fibrosis
Hypersensitivity pneumonitis

Question 8

What is sarcoidosis?

Answer 8

Multisystem granulomatous disease of unknown cause

Question 9

What does sarcoidosis cause?

Answer 9

Non-caseating granulomas, often around pre-existing scars or tattoos

Question 10

Who is sarcoidosis most common in?

Answer 10

Adults under 40
Women>men
Many asymptomatic

Question 11

How is sarcoidosis diagnosed?

Answer 11

History and examination

Chest xray

Question 12

What are the 4 stages of pulmonary involvement of sarcoidosis?

Answer 12

Stage 1- bilateral hilar lymphadenopathy
Stage 2- bilateral hilar lymphadenopathy with pulmonary infiltrates
State 3- pulmonary infiltrates
Stage 4- fibrosis

Question 13

What investigations are done for sarcoidosis?

Answer 13

Pulmonary function tests
Bloods, urinalysis, ECG, TB skin test, eye exam
Bronchoscopy and surgical biopsy

Question 14

How is bronchoscopy useful for the diagnosis of sarcoidosis?

Answer 14

Can include trans bronchial biopsies and endobronchial ultrasound
White patches visible on mucous membranes- granulomas

Question 15

What surgical biopsies can be carried out for sarcoidosis?

Answer 15

Mediastinoscopy

Video assisted thoracoscopic lung biopsy

Question 16

What are the remission rates for sarcoidosis?

Answer 16

Stage 1- 55-90%
Stage 2- 40-70%
State 3- 10-20%
Stage 4- 0% (impossible to reverse fibrosis)

Question 17

What is the treatment of mild sarcoidosis?

Answer 17

Mild disease with no vital organ involvement, normal lung function and few symptoms
-No treatment

Question 18

What is erythema nodosum and the treatment in sarcoidosis?

Answer 18

Inflammation of fat cells under skin

Non steroidal anti inflammatory drugs

Question 19

What is arthralgia and the treatment in sarcoidosis?

Answer 19

Joint pain

Non steroidal anti inflammatory drugs

Question 20

What is the treatment for skin lesions, anterior uveitis and a cough in sarcoidosis?

Answer 20

Topical steroids

Question 21

What is the treatment for cardiac, neurological or eye disease not responding to topical steroids in sarcoidosis?

Answer 21

Systemic steroids

Question 22

What is the treatment for hypercalcaemia in sarcoidosis?

Answer 22

Systemic steroids

Question 23

What is the prognosis for sarcoidosis?

Answer 23

Good, few Caucasians die

10-20% sustain permanent pulmonary or extra pulmonary complications

Question 24

What are the main signs and symptoms of idiopathic pulmonary fibrosis?

Answer 24

Chronic breathlessness and cough
Clubbed fingers
Crackles

Question 25

Who is idiopathic pulmonary fibrosis common in?

Answer 25

60-70 years olds | Men>women

Question 26

What is the treatment for idiopathic pulmonary fibrosis?

Answer 26

Historically only palliative care Oral anti-fibrotic Transplant

Question 27

What is the median survival for idiopathic pulmonary fibrosis?

Answer 27

3 years

Question 28

What is hypersensitivity pneumonitis?

Answer 28

Inflammation of the alveoli

Question 29

What is the interstitial of the lung?

Answer 29

Connective tissue space between the alveoli and capillaries

Question 30

What does interstitial inflammation cause?

Answer 30

Lungs to become stiff

Question 31

What does interstitial lung disease cause?

Answer 31

Reduced lung compliance Low FEV1 and FVC ratio but FEV1/FVC ratio normal Reduced gas transfer V/Q mismatch

Question 32

What is the usual presentation in interstitial lung disease?

Answer 32

Dyspnoea

Question 33

What are the progressive symptoms of interstitial lung disease?

Answer 33

Respiratory failure | Heart failure

Question 34

What is acute inflammation of the lungs associated with?

Answer 34

``` Major trauma Toxic inhalation/chemical injury Circulatory shock Drugs Infection Autoimmune disease Radiation ```

Question 35

What does acute inflammation of the lungs cause?

Answer 35

ARDS

Question 36

What is the evolution of acute inflammation of the lung?

Answer 36

Damage and destruction of endothelial cells causes them to become leaky, causing oedema Causes outport of macromolecules that precipitate and form a hyaline membrane over the endothelium

Question 37

What are the histological features of acute inflammation?

Answer 37

``` Protein rich oedema in alveolar spaces Protein precipitates creating hyaline membranes Fibrin Denuded membranes Epithelial proliferation Fibroblast proliferation Scarring ```

Question 38

What does a granulomatous response to hypersensitivity pneumonitis cause?

Answer 38

Sarcoidosis | Hypersensitivity pneumonitis

Question 39

What is the acute presentation of hypersensitivity pneumonitis?

Answer 39

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

Question 40

What is the chronic presentation of hypersensitivity pneumonitis?

Answer 40

Malaise, dyspnoea, cough | Crackles and some wheeze

Question 41

What is the histopathology of hypersensitivity pneumonitis?

Answer 41

``` Immune complex mediated by type 3 and 4 hypersensitivity reaction Soft centriacinar epithelia granulomata Interstitial pneumonitis Foamy histocytes Bronchiolotis obliterans ```

Question 42

When might usual; interstitial pneumonitis be seen?

Answer 42

Connective tissue diseases Drug reactions Post infection Industrial exposure

Question 43

What is the histopathology of usual interstitial pneumonitis?

Answer 43

Patchy interstitial chronic inflammation Type 2 pneumocyte hyperplasia Smooth muscle and vascular proliferation Proliferating fibroblastic foci

Question 44

What are the clinical signs of usual interstitial pneumonitis?

Answer 44

Dyspnoea, cough | Basal crackles, cyanosis, clubbing

Question 45

Who is usual interstitial pneumonitis common in?

Answer 45

Men>women | Over 50s

Question 46

What investigations are done for usual interstitial pneumonitis?

Answer 46

Chest xray | Pulmonary function test and gas transfer- reduced

Question 47

What is the outcome of usual interstitial pneumonitis?

Answer 47

Increased risk of lung cancer Almost all end in fibrosis Life expectancy= 5 years

Question 48

What are some causes of abnormal pulmonary gas exchange?

Answer 48

Alveolar hypoventilation V/Q imbalance Diffusion impairment Shunt

Question 49

How does alveolar hypoventilation affect pulmonary gas exchange?

Answer 49

Increase PACO2 causes increased PaCO2 and decreased PaO2

Question 50

What is the commonest cause of hypoxaemia clinically?

Answer 50

Low V/Q

Question 51

What are the effects of diffusion impairment on blood gases?

Answer 51

PaCO2 usually not affected | PaO2 maintained at rest but falls significantly on exertion

Question 52

Why does shunt hypoxaemia respond poorly to oxygen?

Answer 52

Oxygenated is already 98% saturated, it is blood not contacting ventilated alveoli causing the problem