Restrictive lung disease Flashcards

1
Q

What is diffuse alveolar damage associated eith?

A
Trauma
Circulatory shock
Chemical exposure/ toxic inhalation
Drugs
Infection
Idiopathic
Radiation
Autoimmune disease
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2
Q

Name the histiological features of DAD.

A
Protein rich oedema
Fibrin
Hyaline membranes
Epithelial proliferation
Fibroblast proliferation
Denuced basement membrane
Scarring in the interstitium and air spaces
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3
Q

Describe how DAD occurs.

A

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

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4
Q

What tests are carried out in the diagnosis of sarcoidosis?

A
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
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5
Q

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

A

No treatment is given it is a self limiting condition.

If there is pain then give non steroidal anti inflammatory drugs

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6
Q

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?

A

Topical steroids

Systemic steroids

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7
Q

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

A
Bronchiectasis
Pnuemothorax
Haemoptysis
Progressive respiratory failure
Aspirgilloma
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8
Q

what is a marker of restriction?

A

vital capacity

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9
Q

what is an abnormal vital capacity?

A

< 80%

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10
Q

what are the pleural causes of lung restriction?

A

pleural effusion
pneumothorax
pleural thickening

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11
Q

what are the bone causes of lung restriction?

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures

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12
Q

what is a muscle cause of lung restriction?

A

motor neurone disease

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13
Q

what are sub diaphragmatic causes of lung restriction?

A

obesity

pregnancy

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14
Q

what are interstitial lung diseases?

A

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

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15
Q

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

A

sarcoidosis

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16
Q

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

A

usual interstitial pneumonitis

idiopathic pulmonary fibrosis

17
Q

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

A

upper zones

18
Q

what is the acute presentation of hypersensitivity pneumonitis?

A
pyrexia
dry cough
myalgia
chills 4-9hrs after Ag exposure
tachypnoea
crackles
wheeze
19
Q

what is the chronic presentation of hypersensitivity pneumonitis?

A

dyspnoea
cough
malaise / low grade illness
crackles wheeze

20
Q

what is the presentation of lofgrens syndrome for sarcoidosis?

A

erythema nudism
acute arthralgia
bilateral hilar lymphadenopathy

21
Q

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

A

soft centriacinar epithelioid granulomata

22
Q

what type of hypersensitivity reaction is hypersensitivity pneumonitis?

A

type III and type IV

23
Q

what conditions might usual interstitial pneumonitis be seen in?

A

connective tissue disorders such as sclerodoma and rheumatoid disease

24
Q

describe the histopathology in usual interstitial pneumonitis.

A

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

25
what are the treatments for UIP?
``` Oral anti fibrotic's such as; - pirfenidone - nintedanib surgical transplantation palliative ```
26
in which restrictive lung disease is fibrin rich oedema found?
diffuse alveolar damage
27
describe the histopathology of hypersensitivity pneumonitis.
soft centriacinar epithelial granulomata interstitial pneumonitis foamy histiocytes bronchiolitis obliterans
28
which restrictive lung disease is primarily around small airways?
hypersensitivity pnuemonitis.
29
in which restrictive lung disease is bronchiolitis obliterates found?
hypersensitivity pneumonitis
30
what are the symptoms of usual interstitial pneumonitis?
cough | dyspnoea
31
what are the signs of usual interstitial pnuemonitis?
clubbing cyanosis bi basal crackles
32
what is the aetiology of UIP?
``` mostly idiopathic connective tissue diseases i.e. sclerodoma and rheumatoid disease drug reaction post infection industrial exposure i.e. asbestos ```
33
what category of people is UIP most commonly found in?
males > 50yrs
34
what is found on examination with someone with hypersensitivity pneumonitis?
crackles