Clinical features and Management of Restrictive Lung Diseases

Question 1

What is the physiological definition of restriction?

Answer 1

Forced vital capacity of less than 80% of predicted normal

Question 2

Describe the differences between the pulmonary function tests between normal, obstructive and restrictive lung

Answer 2

Obstructive

• Larger TLC, RV, FRC
• Smaller VC, TV, IC, ERV

Restrictive

• Smaller TLC, VC, IC, TV, ERV, FRC, RV

Question 3

What is the marker of restrictinon?

Answer 3

Vital capacity - spirometry

Question 4

What are the lung causes of Interstitial lung diseases?

Answer 4

• Idiopathic pulmonary fibrosis
• Sarcoidosis
• Hypersensitivity pneumonitis

Question 5

What are the pleural causes of restrictive lung disease?

Answer 5

Pleural effusion

Pneumothorax

Pleural thickening - asbestos related, pneumonia

Question 6

What are the skeletal causes of restrictive lung diseases?

Answer 6

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures - soreness

Question 7

What are the muscular casues of restrictive lung disease?

Answer 7

Amyotrophic lateral sclerosis

Obesity - sub diaphragmatic - diaphragm can’t fall properly

Pregnancy - sub diaphragmatic

Question 8

What is the interstitium?

Answer 8

Space between alveoli and capillary

Between alveolar epithelium and capillary endothelium

Question 9

What is sarcoidosis?

Answer 9

Multisystem granulomatous disease of unknown cause

Question 10

What is the Histological hallmark of Sarcoidosis?

Answer 10

non-caseating granuloma

Question 11

What are the clinical signs of sarcoidosis?

Answer 11

Erythema nodosum - granulomas often locate to scar tissue

Question 12

Who gets sarcoidosis?

Answer 12

Adults < 40

Women more than men

World-wide

Question 13

How do you investigate sarcoidosis?

Answer 13

History and exam

CXR

Pulmonary function tests

Bloods / urinalysis / ECG / TB skin test / eye exam

Question 14

What would the further assessment of sarcoidosis be?

Answer 14

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

Question 15

What would be the different surgical biopsy options for sarcoidosis

Answer 15

Mediastinoscopy

Video assisted thoracoscopic lung biopsy (VATS)

Question 16

Look at the different stages as presented on these chest X-Rays

Answer 16

Question 17

When would you provide no treatment for Sarcoidosis?

Answer 17

• Mild disease
• no vital organ involvement
• normal lung function
• few symptoms

Question 18

When would you treat sarcoidosis with NSAIDS?

Answer 18

Erythema nodosum / arthralgia

Question 19

When would you treat sarcoidosis with Topical steroids?

Answer 19

Skin lesions / anterior uveitis / cough

Question 20

When would you treat sarcoidosis with Systemic Steroids?

Answer 20

If Cardiac, neurological, eye disease not responding to topical steroids, or in hypercalcaemia

Question 21

What is prognosis?

Answer 21

Few Caucasian’s die (<1%)

10-20% get permanent pulmonary/extra-pulmonary complications

Respiratory:

• Progressive respiratory failure
• Bronchiectasis
• Aspergilloma, haemoptysis, pneumothorax

Question 22

What is the typical presentation for idiopathic pulmonary fibrosis?

Answer 22

• Chronic breathlessness & cough
• Typically 60-70 years old, commoner in men
• Predisposed to lung cancer
• Clubbed & crackles

Question 23

How does fibrotic tissue vary in the lung?

Answer 23

Usually most prominent in peripheral tissue, central tissue is usually unaffected

Worsens as severity of condition increases

Question 24

What are the options for Idiopathic pulmonary fibrosis?

Answer 24

Median survival 3 y.

• refer to ILD clinic

Medical

• OAF (oral anti fibrotic) – slow down progression but does NOT stop it
• palliative care

Surgical

Transplant

Question 25

What initiates hypersensitivity pneumonitis?

Answer 25

Allergens from, pigeons, mould, farms etc