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Respiratory AM > Restrictive lung diseases > Flashcards

Restrictive lung diseases Flashcards

1
Q

What are some lung causes of restrictive lung disease?

A

Interstitial lung diseases

  • idiopathic pulmonary fibrosis
  • sarcoidosis
  • hypersensitivity pneumonitis
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2
Q

What are some pleural causes of restrictive lung disease?

A

Pleural effusion
Pneumothorax
Pleural thickening

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3
Q

What are some skeletal causes of restrictive lung disease?

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty (old TB treatment)
Rib fractures

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4
Q

What is a muscular cause of restrictive lung disease?

A

Amyptrophic lateral sclerosis

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5
Q

What are some sub diaphragmatic causes of restrictive lung disease?

A

Obesity

Pregnancy

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6
Q

What are interstitial lung diseases?

A

Diseases that cause a thickening of the interstitial and can result in pulmonary fibrosis

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7
Q

What are the main 3 types if interstitial lung disease?

A

Sarcoidosis
Idiopathic pulmonary fibrosis
Hypersensitivity pneumonitis

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8
Q

What is sarcoidosis?

A

Multisystem granulomatous disease of unknown cause

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9
Q

What does sarcoidosis cause?

A

Non-caseating granulomas, often around pre-existing scars or tattoos

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10
Q

Who is sarcoidosis most common in?

A

Adults under 40
Women>men
Many asymptomatic

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11
Q

How is sarcoidosis diagnosed?

A

History and examination

Chest xray

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12
Q

What are the 4 stages of pulmonary involvement of sarcoidosis?

A

Stage 1- bilateral hilar lymphadenopathy
Stage 2- bilateral hilar lymphadenopathy with pulmonary infiltrates
State 3- pulmonary infiltrates
Stage 4- fibrosis

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13
Q

What investigations are done for sarcoidosis?

A

Pulmonary function tests
Bloods, urinalysis, ECG, TB skin test, eye exam
Bronchoscopy and surgical biopsy

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14
Q

How is bronchoscopy useful for the diagnosis of sarcoidosis?

A

Can include trans bronchial biopsies and endobronchial ultrasound
White patches visible on mucous membranes- granulomas

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15
Q

What surgical biopsies can be carried out for sarcoidosis?

A

Mediastinoscopy

Video assisted thoracoscopic lung biopsy

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16
Q

What are the remission rates for sarcoidosis?

A

Stage 1- 55-90%
Stage 2- 40-70%
State 3- 10-20%
Stage 4- 0% (impossible to reverse fibrosis)

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17
Q

What is the treatment of mild sarcoidosis?

A

Mild disease with no vital organ involvement, normal lung function and few symptoms
-No treatment

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18
Q

What is erythema nodosum and the treatment in sarcoidosis?

A

Inflammation of fat cells under skin

Non steroidal anti inflammatory drugs

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19
Q

What is arthralgia and the treatment in sarcoidosis?

A

Joint pain

Non steroidal anti inflammatory drugs

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20
Q

What is the treatment for skin lesions, anterior uveitis and a cough in sarcoidosis?

A

Topical steroids

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21
Q

What is the treatment for cardiac, neurological or eye disease not responding to topical steroids in sarcoidosis?

A

Systemic steroids

22
Q

What is the treatment for hypercalcaemia in sarcoidosis?

A

Systemic steroids

23
Q

What is the prognosis for sarcoidosis?

A

Good, few Caucasians die

10-20% sustain permanent pulmonary or extra pulmonary complications

24
Q

What are the main signs and symptoms of idiopathic pulmonary fibrosis?

A

Chronic breathlessness and cough
Clubbed fingers
Crackles

25
Q

Who is idiopathic pulmonary fibrosis common in?

A

60-70 years olds

Men>women

26
Q

What is the treatment for idiopathic pulmonary fibrosis?

A

Historically only palliative care
Oral anti-fibrotic
Transplant

27
Q

What is the median survival for idiopathic pulmonary fibrosis?

A

3 years

28
Q

What is hypersensitivity pneumonitis?

A

Inflammation of the alveoli

29
Q

What is the interstitial of the lung?

A

Connective tissue space between the alveoli and capillaries

30
Q

What does interstitial inflammation cause?

A

Lungs to become stiff

31
Q

What does interstitial lung disease cause?

A

Reduced lung compliance
Low FEV1 and FVC ratio but FEV1/FVC ratio normal
Reduced gas transfer
V/Q mismatch

32
Q

What is the usual presentation in interstitial lung disease?

A

Dyspnoea

33
Q

What are the progressive symptoms of interstitial lung disease?

A

Respiratory failure

Heart failure

34
Q

What is acute inflammation of the lungs associated with?

A 
Major trauma
Toxic inhalation/chemical injury
Circulatory shock
Drugs
Infection
Autoimmune disease
Radiation
35
Q

What does acute inflammation of the lungs cause?

A

ARDS

36
Q

What is the evolution of acute inflammation of the lung?

A

Damage and destruction of endothelial cells causes them to become leaky, causing oedema
Causes outport of macromolecules that precipitate and form a hyaline membrane over the endothelium

37
Q

What are the histological features of acute inflammation?

A 
Protein rich oedema in alveolar spaces
Protein precipitates creating hyaline membranes
Fibrin
Denuded membranes
Epithelial proliferation
Fibroblast proliferation
Scarring
38
Q

What does a granulomatous response to hypersensitivity pneumonitis cause?

A

Sarcoidosis

Hypersensitivity pneumonitis

39
Q

What is the acute presentation of hypersensitivity pneumonitis?

A

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

40
Q

What is the chronic presentation of hypersensitivity pneumonitis?

A

Malaise, dyspnoea, cough

Crackles and some wheeze

41
Q

What is the histopathology of hypersensitivity pneumonitis?

A 
Immune complex mediated by type 3 and 4 hypersensitivity reaction
Soft centriacinar epithelia granulomata
Interstitial pneumonitis
Foamy histocytes
Bronchiolotis obliterans
42
Q

When might usual; interstitial pneumonitis be seen?

A

Connective tissue diseases
Drug reactions
Post infection
Industrial exposure

43
Q

What is the histopathology of usual interstitial pneumonitis?

A

Patchy interstitial chronic inflammation
Type 2 pneumocyte hyperplasia
Smooth muscle and vascular proliferation
Proliferating fibroblastic foci

44
Q

What are the clinical signs of usual interstitial pneumonitis?

A

Dyspnoea, cough

Basal crackles, cyanosis, clubbing

45
Q

Who is usual interstitial pneumonitis common in?

A

Men>women

Over 50s

46
Q

What investigations are done for usual interstitial pneumonitis?

A

Chest xray

Pulmonary function test and gas transfer- reduced

47
Q

What is the outcome of usual interstitial pneumonitis?

A

Increased risk of lung cancer
Almost all end in fibrosis
Life expectancy= 5 years

48
Q

What are some causes of abnormal pulmonary gas exchange?

A

Alveolar hypoventilation
V/Q imbalance
Diffusion impairment
Shunt

49
Q

How does alveolar hypoventilation affect pulmonary gas exchange?

A

Increase PACO2 causes increased PaCO2 and decreased PaO2

50
Q

What is the commonest cause of hypoxaemia clinically?

A

Low V/Q

51
Q

What are the effects of diffusion impairment on blood gases?

A

PaCO2 usually not affected

PaO2 maintained at rest but falls significantly on exertion

52
Q

Why does shunt hypoxaemia respond poorly to oxygen?

A

Oxygenated is already 98% saturated, it is blood not contacting ventilated alveoli causing the problem

Respiratory AM (10 decks)

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