Restrictive Lung Disease

Question 1

Presentation of RLD

Answer 1

Abnormal CXR
Dyspnoea (exertion, progressing to rest)
T1 respiratory failure
HF

Question 2

Which RLD is the most common cause of end stage honeycomb disease?

Answer 2

Usual Interstitial Pneumonitis

Question 3

Presentation of Diffuse lung disease (ARDS)

Answer 3

Abnormal CXR (fewer ribs visible)
Dyspnoea
T1 respiratory failure
HF

Question 4

Restrictive lung diseases mean what?

Answer 4

Reduced lung compliance

Question 5

FEV1 and FEV1/FVC in RLD?

Answer 5

FEV1 decreased

FEV1/FVC normal or increased

Question 6

What is Diffuse Alveolar damage associated with?

Answer 6

Trauma
Chemical injury
Circulatory shock
Drugs
Infection
Autoimmune
Radiation

Question 7

How does DADS evolve to fibrosis?

Answer 7

Vascular damage
Proteinous oedema
Hyaline membrane formation
Denuded basement membrane
Epithelial/fibroblast proliferation
Inflammation --> scarring

Question 8

Histological features of DADS

Answer 8

Protein rich oedema (not in alveolar space)
Fibrin
Hyaline membranes expanded
Cell proliferation

Question 9

Histopathology of Sarcoidosis?

Answer 9

Giant cell granulomas (non-caseating)
Little lymphoid infiltrate
Variable fibrosis

Question 10

Sarcoidosis more commonly effects who?

Answer 10

Afro-americans (US)
Females
Young

Question 11

Most common organs involved in sarcoidosis?

Answer 11

Lymph nodes
Lung
Spleen
Liver

Question 12

How does sarcoidosis present?

Answer 12

Young adult 
Arthralgia, erythema nodosum
Bilateral hilar lymphadenopathy
Incidental finding
SoB, cough, abnormal CXR

Question 13

How is sarcoidosis diagnosed?

Answer 13

Clinical and imaging findings
Serum Ca++ and ACE
Biopsy

Question 14

How is sarcoidosis treated?

Answer 14

Corticosteroids

Question 15

Most common causes of Hypersensitivity pneumonitis?

Answer 15

Thermophilic actinomyces - farmers
Bird/animal proteins
Fungi - aspergillus

Question 16

Acute presentation of hypersensitivity pneumonitis?

Answer 16

Fever, dry cough, myalgia
Chills after exposure
Crackles, tachypnoea, wheeze
Antibody

Question 17

Chronic presentation of hypersensitivity pneumonitis?

Answer 17

Insidious
Malaise, SOB, cough
Low grade illness
Crackles and some wheeze

Question 18

What is important in order to differentiate HP from pneumonia?

Answer 18

History! - exposure/living

Question 19

Histopathology of Hypersenstiivity pneumonitis?

Answer 19

Type 3 and 4 hypersensitivity
Soft centriacinar granulomata
Upper zone disease

Question 20

Usual interstitial pneumonitis is often seen in which conditions?

Answer 20

Connective tissue disease
Drug reaction
Post infection
Industrial exposure - asbestos

Question 21

Histopathology of UP

Answer 21

Chronic interstitial inflammation
Type 2 pneumocyte hyperplasia
Smooth muscle and vascular proliferation

Question 22

Idiopathic pulmonary fibrosis presents how?

Answer 22

Elderly (M>F)
Dyspnoea, Cough
Basal crackles, 
Cyanosis
Clubbing

Question 23

Investigations for UIP? What will likely be seen?

Answer 23

CXR - cysts, ‘ground glass’
PFT
Gas transfer (reduced)

Question 24

Treatment for interstitial lung disease

Answer 24

Anti-angiogenic agents

Steroids

Question 25

Where is air flow turbulent?

Answer 25

Bifurcations

Question 26

Type 1 respiratory failure presents with what volumes?

Answer 26

PaO2 <8kPa

normal or low PaCO2

Question 27

Type 1 respiratory failure presents with what volumes?

Answer 27

PaCO2 >6.5kPa

PaO2 usually low

Question 28

What abnormal states are associated with hypoxaemia?

Answer 28

Alveolar Hypoventilation
Shunt
V/Q imbalance
Diffusion impairment

Question 29

How is a fall in PaO2 due to hypoventilation fixed?

Answer 29

Raising FIO2

Question 30

What is a normal V/Q?

Answer 30

0.8