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Respiratory System - exam style qs > Restictive lung disease - sarcoidosis, idiopathic pulmonary fibrosis, diffuse alveolar damage syndrome, hypersensitivity pneumonitis > Flashcards

Restictive lung disease - sarcoidosis, idiopathic pulmonary fibrosis, diffuse alveolar damage syndrome, hypersensitivity pneumonitis Flashcards

1
Q

What part of the lung is affected by restrictive lung disease

A

the interstitium - connective tissue space around airways and vessels and space between basement membranes of alveolar walls

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2
Q

Characteristics typical of restrictive interstitial lung disease

A

Reduced lung compliance - stiff lungs
Low spirometry
Reduced gas transfer
Ventilation/perfusion imbalance

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3
Q

Complications of restrictive lung disease

A

hypoxic cor pulmonale, heart failure, type 1 respiratory failure

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4
Q

Restrictive lung disease is interstitial inflammation. What, in very general terms, causes the inflammatory response?

A

Parenchymal (interstitial) lung injury

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5
Q

What is the name of the syndrome relating to an acute inflammatory response

A

Diffuse alveolar damage syndrome

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6
Q

Aetiology of diffuse alveolar damage syndrome?

A 
IDIOPATHIC
Major trauma
Chemical injury/toxic inhalation
Circulatory shock
Drugs
Infection
Auto(immune) disease
Radiation
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7
Q

What happens in the exudative stage of diffuse alveolar damage syndrome

A

Oedema

Hyaline membrane formation

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8
Q

What happens in the proliferative stage of diffuse alveolar damage syndrome

A

Interstitial inflammation

Interstitial fibrosis

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9
Q

Histological features of DADS

A 
protein rich oedema
fibrin
hyaline membranes
denuded basement membranes
epithelial proliferation
fibroblast proliferation
scarring
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10
Q

Granulomatous responses are a sub type of chronic response to lung injury. What 2 diseases do you need to know for it?

A

Sarcoidosis

Hypersensitivity pneumonitis

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11
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder of unknown aetiology

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12
Q

Is sarcoidosis caesating?

A

No (but TB is)

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13
Q

Histopathology of sarcoidosis

A

epithelioid and giant cell granulomas
little lymphoid infiltrate
variable associated fibrosis

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14
Q

Who does sarcoidosis normally affect?

A

Young adults

Female more than male

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15
Q

Presentation of sarcoidosis

A 
acute arthralgia (knee pain)
erythema nodosum (rash)
bilateral hilar lymphadenopathy
incidental abnormal CXR
dyspnoea
cough
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16
Q

Prognosis of sarcoidosis

A

Most resolve

17
Q

Prognosis of DADS

A

fatal

18
Q

Investigations with sarcoidosis

A

CXR
Blood test - serum calcium and ACE
Biopsy

19
Q

Antigens of hypersensitivity pneumonitis

A

Thermophilic actinomycetes
Bird/animal proteins
Fungi
Chemicals

20
Q

Acute presentation of hypersensivity pneumonitis

A

Fever, dry cough, myalgia
Chills 4-9 hours after exposure
Crackles, tachypnoea, wheeze
Precipitating antibody

21
Q

Chronic presentation of hypersensitivity pneumonitis

A

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

22
Q

Where in the lung is hypersensitivity pneumonitis common?

A

Upper zone

23
Q

What type of hypersensitivity reaction is in hypersensitivity pneumonitis?

A

Type 3 and 4

24
Q

Aetiology of interstitial pneumonitis (v bad one)

A

IDIOPATHIC or cryptogenic

Connective tissue diseases
Drug reaction
Post infection
Industrial exposure

25
Q

3 things making up the histopathology of interstitial pneumonitis

A

Patchy interstitial chronic inflammation
Type II pneumocyte hyperplasia
Smooth muscle and vascular proliferation

26
Q

Typical patient having idiopathic pulmonary fibrosis

A

elderly
male
progressive disease

27
Q

What do all three chronic responses go to if they do not resolve (or haven’t died already)

A

Fibrosis and end stage honeycomb lung

28
Q

Definition of restriction

A

FVC <80% predicted

29
Q

What are the lung causes of restriction (3 to remember)

A

sarcoidosis
idiopathic pulmonary fibrosis
hypersensitivity pneumonitis

30
Q

Pleural causes for restriction

A

pleural effusion
pneumothorax
pleural thickening

31
Q

Skeletal causes for restriction

A

kyphoscoliosis
ankylosing spondylitis
thoracoplasaty
rib fractures

32
Q

Muscle causes for restriction

A

amyotrophic lateral sclerosis

33
Q

sub-diaphragmatic causes for restriction

A

obesity

pregnancy

34
Q

Investigations for sarcoidosis

A 
CXR
Pumonary function test
Bloods
Urinalysis
ECG
TB skin test
eye exam
HR CT
Surgical biopsy
35
Q

Treatment of sarcoidosis

A

None if mild
NSAIDS
Topical steroids
Systemic steroids

36
Q

Treatment for idiopathic pulmonary fibrosis

A

Oral antifibrotic
Palliative treatment
Transplant

Respiratory System - exam style qs (13 decks)

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