Week 5 - Clinical Features and Management of Restrictive Lung Disease

Question 1

What is the physiological definition of restrictive lung disease?

Answer 1

FVC<80% of normal. expected value is calculated based on age, height, sex . in graph, every area is restricted. tidal volume, TLC and RV all reduce

Question 2

What 3 diseases do we focus on?

Answer 2

sarcoidosis, Idiopathic Pulmonary Fibrosis and hypersensitivity pneumonitis

Question 3

What are pleural causes of restrictive lung disease?

Answer 3

pleural effusion, pleural thickening (asbestos), pneumothorax

Question 4

What are skeletal causes of restrictive lung disease?

Answer 4

kyphoscoliosis, rib fractures, ankylosis spondylitis (inflamed spine), thoracoplasty (ribs removed, rare)

Question 5

What are muscular causes of restrictive lung disease?

Answer 5

amyotrophic lateral sclerosis - inability to expand lungs

Question 6

What are sub-diaphragmatic causes of restrictive lung disease?

Answer 6

• obesity - diaphragm impaired due to fat.
• Pregnancy - feel breathless

Question 7

What is an overview of sarcoidosis and its main symptoms?

Answer 7

idiopathic, causing non-ceasiating granulomas. multi-system disorder causing erythema nodosum, uveitis, pink granulomatous lesions on skin

Question 8

How do you investigate sarcoidosis?

Answer 8

• history,
• exam,
• CXR.
• pulmonary function tests,
• bloods,
• urinalysis,
• ECG,
• TB skin test,
• eye exam.
• Bronchoscopy and EBUS for view and samples.

Question 9

How do you treat sarcoidosis?

Answer 9

by symptoms. if mild and no organ involvement, no treatment. give topical steroids for skin lesions and uveitis. give systemic steroids for life threatening disease.

Question 10

What is the prognosis of sarcoidosis?

Answer 10

usually ok.
In 10-20% patients it can lead to progressive resp. failure, bronchiectasis, aspergilloma, haemoptysis, pneumothorax

Question 11

What is IPF?

Answer 11

idiopathic pulmonary fibrosis. forms fibroblastic collagen focus’s.

Question 12

What does IPF look like of a CT scan?

Answer 12

edges have honeycombing and traction bronchiectasis seen by fibrosis stretching bronchioles

Question 13

What is the median survival and treatment for IPF?

Answer 13

3 years. treat with oral anti-fibrotic or palliative.

Question 14

What are the symptoms of idiopathic pulmonary fibrosis? Who gets it?

Answer 14

• 60-70yrs, usually male
• chronic breathlessness and cough
• crackles
• clubbed
• doesnt get better with antibiotics

Question 15

What causes hypersensitivity Pneumonitis?

Answer 15

Budgies, pigeons, malt whiskey factory

Question 16

What are 4 stages of sarcoidosis?

Answer 16

Stage 1-4 with decreasing remission rates

Stage 1 - bulky glands and paratracheal enlargement xray

Stage 2 - spotty lungs as well as lymph

Stage 3 - interstitial change

Stage 4 - fibrosis. Irreversible