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

Look

Answer 2

Question 3

What is the marker of restrictino?

Answer 3

Vital capacity - spirometry

Question 4

What are the lung causes of Interstitial lung diseases?

Answer 4

Question 5

What are the possible routes of restriction?

Answer 5

Question 6

What are the pleural causes of restrictive lung disease?

Answer 6

Pleural effusion

Pneumothorax

Pleural thickening - asbestos related, pneumonia

Question 7

What are the skeletal causes of restrictive lung diseases?

Answer 7

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures - soreness

Question 8

What are the muscular casues of restrictive lung disease?

Answer 8

Amyotrophic lateral sclerosis

Obesity - sub diaphragmatic - diaphragm can’t fall properly

Pregnancy - sub diaphragmatic

Question 9

What is the interstitium?

Answer 9

Question 10

What is sarcoidosis?

Answer 10

Multisystem granulomatous disease of unknown cause

Question 11

What is the Histological hallmark of Sarcoidosis?

Answer 11

non-caseating granuloma

Question 12

What are the clinical signs of sarcoidosis?

Answer 12

Erythema nodosum - granulomas often locate to scar tissue

Question 13

Who gets sarcoidosis?

Answer 13

Question 14

How do you investigate sarcoidosis?

Answer 14

History and exam

CXR

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

Pulmonary function tests

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

Surgical Biopsies (Mediastinoscopy, Video assisted thoracoscopic lung biopsy (VATS))

Question 15

Look at chest x - rays

Answer 15

.

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

Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

Question 21

What is prognosis?

Answer 21

Few Caucasian’s die (<1%)

10-20% sustain permanent pulmonary or extra-pulmonary complications

Respiratory

»Progressive respiratory failure

»Bronchiectasis

»Aspergilloma, haemoptysis, pneumothorax

Question 22

What is the typical presentation for idiopathic pulmonary fibrosis?

Answer 22

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 the condition increases

Question 24

What are the options for Idiopathic pulmonary fibrosis?

Answer 24

Question 25

What initiates hypersensitivity pneumonitis?

Answer 25

Allergens from things like, pigeons, mould, farms etc