Restrictive lung disease

Question 1

What is restrictive interstitial lung disease

Answer 1

A large group of disorders that caues progressive scarring/thickening of the interstitium of the lung that prevents it from fully expanding with air. The scarring is irreversible.

Question 2

What does the patient present with in Restrictive lung disease

Answer 2

S.O.B at rest or on exertion (MRC Dyspnoea Scale)

Dry cough

Question 3

What are the clinical signs of restrictive lung disease

Answer 3

Type 1 respiratory failure (cyanotic)
Abnormal CXR
Low FEV1 and FVC (normalish ratio)
increased respiratory rate

Question 4

How does restrictive lung disease cause Hypoxaemia

Answer 4

Reduced gas transfer
Ventilation/perfusion imbalance
Reduced compliance

Question 5

What happens in end stage restrictive lung diease

Answer 5

Fibrosis and End-stage honeycombing of the lung.

Question 6

What is the common acute response to interstitial lung injury called?

Answer 6

Diffuse alveolar damage

Question 7

What typically causes Diffuse alveolar damage

Answer 7

Trauma
Chemical/toxic inhalation 
Circulatory shock
Drugs
Infection 
Auto-immune response

Question 8

What happens to the lungs after diffuse alveolar damage

Answer 8

Its swells with protein rich oedema
Interstitial inflammation and fibrosis (scarring)
deposition of hyaline membranes causing alveolar collapse and non-compliant lungs

Question 9

What are the 2 categories of response to chronic restrictive interstitial lung disease

Answer 9

Granulomatous

Usual Interstitial Pneumonitis

Question 10

What are the granulomatous responses to interstitual lung injury

Answer 10

Sarcoidosis

Hypersensitivity pneumonitis

Question 11

What is sarcoidosis

Answer 11

Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomas.

Question 12

How long does it take to recover from sarcoidosis

Answer 12

usually around 1-2 years

Question 13

What are the clinical signs of Sarcoidosis

Answer 13

Acute athralgia
Erythema nodosum (swollen fat under skin, (usually legs))
Bilateral hilar lymphadenopathy
Tender reddish bumps or patches on the skin

Question 14

What investigations should you do if suspect sarcoidosis

Answer 14

X-ray
serum ca and angiotensin converting enzyme
EBUS (sample)

Question 15

what causes sarcoidosis

Answer 15

unknown

Question 16

What age group and sex is more succeptible to Sarcoidosis

Answer 16

Females

20-40 years

Question 17

What does a sarcoidosis positive Xray look like?

Answer 17

opaque

Question 18

What can be prescribed to treat sarcoidosis

Answer 18

Corticosteroids

Question 19

What is hypersensitivity pneumonitis

Answer 19

Hypersensitivity to inhaled organic dusts causing inflammation of the alveoli. It can lead to respiratory failure by reducing gas transfer.

Question 20

What does the patient present with when they have hypersensitivity pneumonitis

Answer 20

Fever
Dry cough
Increased breathing rate
Wheeze

Question 21

What are the clinical findings of hypersensitivity pneumonitis

Answer 21

Crackles

Question 22

What is Usual Interstitial pneumonitis

Answer 22

Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the supporting framework (interstitium) of the lung.

Question 23

What does the patient present with in Usual interstitial pneumonitis

Answer 23

S.O.B
Cough 
Basal Crackles
Cyanosis 
Clubbing

Question 24

What is a marker of lung restriction and reduces in people with restrictive interstitial lung disease and how do you measure it?

Answer 24

Vital capacity

Question 25

What areas commonly get Sarcoidosis

Answer 25

The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver, heart, and brain. Any organ, however, can be affected.

Question 26

How do you investigate possible sarcoidosis

Answer 26

Pulmonary function tests Bloods / urinalysis / ECG / TB skin test / eye exam EBUS

Question 27

What are the stages of Sarcoidosis

Answer 27

1 - nodal 2 - nodal and parenchymal 3 - nodal, parenchymal and 4 - honey comb (massive fibrosis) - lethal

Question 28

How should you treat mild Sarcoidosis

Answer 28

No treatment

Question 29

How should you treat Erythema Nodosum and athralgia

Answer 29

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Question 30

How should you treat Skin lesions / anterior uveitis / cough

Answer 30

Topical steroids

Question 31

How should you treat Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

Answer 31

Systematic steroids

Question 32

What are the presenting symptoms for Idiopathic pulmonary fibrosis

Answer 32

Chronic breathlessness & cough Typically 60-70 years old, commoner in men Failed prescription for “LVF” or infection Clubbed & crackles

Question 33

What is the median survival rate for Idiopatathic pulmonary fibrosis

Answer 33

3 years

Question 34

What medical treatment can you give to people with ideopathic pulmonary fibrosis

Answer 34

``` OAF (oral anti-fibrotic) - Pirfenidone, Nintedanib Lung transplant (best prognosis) ```

Question 35

What are the most common restrictive lung diseases

Answer 35

sarcoidosis ideopathetic pulmonary fibrosis Hypersenstivity pneumontis