Restrictive Lung Diseases

Question 1

Which structure(s) are mainly damaged in interstitial lung disease?

Answer 1

Alveolar walls and lumen

Causes impaired gas exchange

Question 2

CO2 and O2 exchange is impaired. True/False?

Answer 2

False

CO2 exchange is unimpaired as it is very soluble

Question 3

How can consolidation of alveolar spaces arise?

Answer 3

Infection (pneumonia)
Infarction (PE)
BOOP (none of above)

Question 4

What is sarcoidosis?

Answer 4

Non-caseating granulomatous disease (Type IV hypersensitivity) of unknown aetiology

Question 5

Sarcoidosis is less common in smokers. True/False?

Answer 5

True!

Question 6

List some clinical signs of sarcoidosis

Answer 6

Erythema nodosum
Bilateral hilar lymphadenopathy
Arthritis
Fever
Lung infiltrates
Multi-system damage

Question 7

Is treatment given for sarcoidosis?

Answer 7

Oral steroids and immunosuppressives ONLY IF vital organs are being affected/chronic
If acute, recommend NSAIDs and bed rest

Question 8

Symptoms of EAA occur rapidly. True/False?

Answer 8

False

Typically several hours after exposure

Question 9

How is EAA treated?

Answer 9

Antigen avoidance

Oral steroids if very breathless

Question 10

What is the most common ILD?

Answer 10

Idiopathic pulmonary fibrosis

Question 11

What is the proposed aetiology of idiopathic PF?

Answer 11

Imbalance in fibrotic repair, resulting in scarring of the lung

Question 12

Name some drugs which can cause IPF

Answer 12

Amiodarone, bleomycin, methotrexate

Question 13

List some clinical signs of IPF (different from those of a typical ILD)

Answer 13

Progressive breathlessness
Clubbing
Bilateral fine inspiratory crackles

Question 14

What is pneumoconiosis?

Answer 14

ILD caused by inhalation of occupational dusts e.g. asbestos

Question 15

Describe the early and late stages of ILD

Answer 15

Early: alveolitis (inflammatory infiltrates)
Late: scarring

Question 16

Lung tissue becomes fibrous in IPF. What is the physiological consequence of this?

Answer 16

Reduced compliance (not as elastic)

Question 17

Define asbestosis

Answer 17

Fibrosis due to asbestos exposure

Question 18

IPF is commoner in females. True/False?

Answer 18

False

Commoner in males

Question 19

What is heard on auscultation in IPF?

Answer 19

Fine bilateral end-inspiratory crackles

Question 20

What is extrinsic allergic alveolitis?

Answer 20

Inflammation in airways due inhalation of foreign antigens, usually from animals provokes a type 3 hypersensitivity reaction

Question 21

Smokers have increased risk of EAA. True/False?

Answer 21

False

Decreased risk!

Question 22

What is heard on auscultation in EAA?

Answer 22

Coarse end-inspiratory crackles

Question 23

List some features of interstitial lung disease…

Answer 23

SOB on exertion
Non-productive cough
NO WHEEZE
Abnormal CXR
Restrictive spirometry

Question 24

How are ILDs investigated? Blood tests for sarcoidosis and EAA?

Answer 24

CXR/CT of lungs
Tissue biopsy
PFTs
Blood tests - 
INCREASED ACE AND CA LEVELS IN SARCOIDOSIS
INCREASED IGG ANTIBODIES IN EAA

Question 25

List some symptoms of EAA (different from those of a typical ILD)

Answer 25

Fever Rigors Myalgia

Question 26

How is IPF treated?

Answer 26

Palliative care (not steroids/immunosuppressants)

Question 27

ILDs typically affect which area of the lung? What is the exception to this rule?

Answer 27

Apex of lung | Asbestosis - base of lung

Question 28

What is asbestosis associated with?

Answer 28

Increased risk of bronchial adenocarcinoma Pleural plaques Malignant mesothelioma

Question 29

Cystic fibrosis is a congenital cause of...

Answer 29

Bronchiectasis

Question 30

List some features of CF

Answer 30

``` Weight loss Salty sweat Failure to thrive Recurrent lung infection Male infertility (abscence of vas deferens) Nasal polyps and filled sinuses ```

Question 31

Describe the pathophysiology of CF

Answer 31

AR mutation in CFTR gene that codes for the chlorine channel - leads to increased sodium absorption with water ---> dryness, blocked ducts, impaired mucosal defence

Question 32

List some tests used in the diagnosis of CF

Answer 32

``` Faecal elastase Salty sweat Genetic screening Sputum culture CXR Abdo US Spirometry ```

Question 33

Which organs are affected by CF?

Answer 33

Pancreas - exocrine (digestion) and endocrine (sugars) failure Colon - constipation Liver - portal hypertension Gallbladder

Question 34

Define stridor

Answer 34

Inspiratory wheeze due to large airway obstruction

Question 35

List the main causes of stridor

Answer 35

``` Neoplasms Anaphylaxis Goitre/angioneurotic oedema Trauma Infection Foreign body ```

Question 36

List some investigations used for stridor

Answer 36

``` Laryngoscopy Bronchoscopy CXR CT Thyroid scan ```

Question 37

Define obstructive sleep apnoea

Answer 37

Intermittent upper airway pharyngeal collapse causing episodes during sleep

Question 38

List some risk factors for sleep apnoea

Answer 38

Enlarged tonsils Obesity Acromegaly Hypothyroidism

Question 39

Give the two main consequences of sleep apnoea

Answer 39

Pulmonary hypertension | Type 2 respiratory failure

Question 40

How is sleep apnoea diagnosed?

Answer 40

Snoring Raised Epworth score Overnight sleep study

Question 41

How is sleep apnoea treated?

Answer 41

Remove cause CPAP (continuous positive airway pressure) via nasal mask Mandibular advancement device Surgery to relieve obstruction

Question 42

What are some symptoms of obstructive sleep apnoea?

Answer 42

Loud snoring Daytime sleepiness Headache

Question 43

What is the criteria for diagnosing OSA?

Answer 43

If more than 10-15 apnoeas in any 1hr of sleep