Restrictive lung disease

Question 1

What is diffuse alveolar damage associated eith?

Answer 1

Trauma
Circulatory shock
Chemical exposure/ toxic inhalation
Drugs
Infection
Idiopathic
Radiation
Autoimmune disease

Question 2

Name the histiological features of DAD.

Answer 2

Protein rich oedema
Fibrin
Hyaline membranes
Epithelial proliferation
Fibroblast proliferation
Denuced basement membrane
Scarring in the interstitium and air spaces

Question 3

Describe how DAD occurs.

Answer 3

Vascular damage causes oedema
Oedema is rich in protein including fibrin
Proteinaceous cosgulation deposits creates hyaline membranes which causes inflammation
The inflammation leads to fibrosis within the interstitium and alveolar walls

Question 4

What tests are carried out in the diagnosis of sarcoidosis?

Answer 4

Test for increased serum calcium
Test for increased serum ACE levels
TB skin test to rule out TB 
Biopsy
Urinalysis
Bloods

Further investigation:

• bronchoscopy and transthoracic biopsy
• mediastinoscopy
• video assisted thoracic lung biopsy
• endobronchial ultrasound

Question 5

If a patient presents with lofgrens syndrome which is indicates sarcoidosis what is the treatment?

Answer 5

No treatment is given it is a self limiting condition.

If there is pain then give non steroidal anti inflammatory drugs

Question 6

If a patient presents with skin lesions or anterior uvietis which indictaes sarcoidosis, what is the treatment?
And if the treatment doesnt work what other treatment can be given?

Answer 6

Topical steroids

Systemic steroids

Question 7

10-20% of patients with sarcoidosis sustain permenant pulmonary or extra pulmonary complications. Name some of these:

Answer 7

Bronchiectasis
Pnuemothorax
Haemoptysis
Progressive respiratory failure
Aspirgilloma

Question 8

what is a marker of restriction?

Answer 8

vital capacity

Question 9

what is an abnormal vital capacity?

Answer 9

< 80%

Question 10

what are the pleural causes of lung restriction?

Answer 10

pleural effusion
pneumothorax
pleural thickening

Question 11

what are the bone causes of lung restriction?

Answer 11

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

Question 12

what is a muscle cause of lung restriction?

Answer 12

motor neurone disease

Question 13

what are sub diaphragmatic causes of lung restriction?

Answer 13

obesity

pregnancy

Question 14

what are interstitial lung diseases?

Answer 14

diseases which cause thickening of the interstitial and can result in pulmonary fibrosis

Question 15

what restive lung disease is more common in females (< 40yrs) ?

Answer 15

sarcoidosis

Question 16

what restrictive lung disease is more common in men > 50 yrs?

Answer 16

usual interstitial pneumonitis

idiopathic pulmonary fibrosis

Question 17

what region is hypersensitivity pneumonitis likely to occur in the lungs?

Answer 17

upper zones

Question 18

what is the acute presentation of hypersensitivity pneumonitis?

Answer 18

pyrexia
dry cough
myalgia
chills 4-9hrs after Ag exposure
tachypnoea
crackles
wheeze

Question 19

what is the chronic presentation of hypersensitivity pneumonitis?

Answer 19

dyspnoea
cough
malaise / low grade illness
crackles wheeze

Question 20

what is the presentation of lofgrens syndrome for sarcoidosis?

Answer 20

erythema nudism
acute arthralgia
bilateral hilar lymphadenopathy

Question 21

what are the characteristics of the granulomata found in hypersensitivity pneumonitis?

Answer 21

soft centriacinar epithelioid granulomata

Question 22

what type of hypersensitivity reaction is hypersensitivity pneumonitis?

Answer 22

type III and type IV

Question 23

what conditions might usual interstitial pneumonitis be seen in?

Answer 23

connective tissue disorders such as sclerodoma and rheumatoid disease

Question 24

describe the histopathology in usual interstitial pneumonitis.

Answer 24

patchy interstitial chronic inflammation
type II pneumocyte hyperplasia
smooth muscle and vascular proliferation
proliferating fibroblastic foci

Question 25

what are the treatments for UIP?

Answer 25

Oral anti fibrotic's such as;
- pirfenidone
- nintedanib
surgical transplantation
palliative

Question 26

in which restrictive lung disease is fibrin rich oedema found?

Answer 26

diffuse alveolar damage

Question 27

describe the histopathology of hypersensitivity pneumonitis.

Answer 27

soft centriacinar epithelial granulomata
interstitial pneumonitis
foamy histiocytes
bronchiolitis obliterans

Question 28

which restrictive lung disease is primarily around small airways?

Answer 28

hypersensitivity pnuemonitis.

Question 29

in which restrictive lung disease is bronchiolitis obliterates found?

Answer 29

hypersensitivity pneumonitis

Question 30

what are the symptoms of usual interstitial pneumonitis?

Answer 30

cough

dyspnoea

Question 31

what are the signs of usual interstitial pnuemonitis?

Answer 31

clubbing
cyanosis
bi basal crackles

Question 32

what is the aetiology of UIP?

Answer 32

mostly idiopathic
connective tissue diseases i.e. sclerodoma and rheumatoid disease
drug reaction
post infection
industrial exposure i.e. asbestos

Question 33

what category of people is UIP most commonly found in?

Answer 33

males > 50yrs

Question 34

what is found on examination with someone with hypersensitivity pneumonitis?

Answer 34

crackles