Interstitial Lung Disease Flashcards

1
Q

What is the ‘interstitial space’?

When should it appear and what might it contain?

A

A potential space between alveolar cells and the capillary basement membrane.

It is only apparent in disease states and may contain fibrous tissue, cells or fluid

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2
Q

Which structures does interstitial disease affect?

A

Acini + alveolar lumen, bronchioles + bronchiolar lumen and cell types; epithelial, endothelial, macrophages, inflamm cells, etc.

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3
Q

What does interstitial lung disease lead to?

A

Temporary or permanent changes in the interstitial tissues interferes with diffusion of gases and changes the mechanics of breathing

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4
Q

Is interstitial lung disease obstructive or restrictive?

A

Fibrous tissue in the interstitium makes the lungs less compliant - producing a restrictive deficit

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5
Q

What are the 5 types of interstitial lung disease?

A
  1. Occupational
  2. treatment related
  3. CT disease
  4. Immunological
  5. Idiopathic
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6
Q

How long has the patient had interstitial lung disease if they have the acute, sub-acute or chronic version?

A

Acute: days-weeks
Sub-acute: weeks- months
Chronic: months-years

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7
Q

How does interstitial lung disease present?

A
  1. progressive breathlessness
  2. dry cough
  3. extra-pulmonary symptoms: fever, joint pain, lethargy, etc.
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8
Q

What are you looking for in a history that might complete your diagnosis?

A
  1. Onset of symptoms: duration of symptoms
  2. Past medical history: history of CT disease, malignancy, inflamm. bowel disease
  3. Smoking and occupational and environmental exposure
  4. Prior medication
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9
Q

How will a patient with interstitial lung disease appear on examination?

A
  1. cyanosis
  2. tachycardia
  3. tachypnoea
  4. finger clubbing
  5. fine end inspiratory crackles
  6. Signs of R heart failure like peripheral oedema
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10
Q

How might you investigate a patient with ILD?

A
  1. X ray
  2. Lung function test
  3. HRCT
  4. May need a surgical lung biopsy
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11
Q

What chemicals/molecules could cause occupational/environmental exposure ILD?

A
  1. Inhaled organic dust; stone and coal dust and asbestos fibres
  2. Silicates
  3. Carbon
  4. Metals
  5. Inhaled agents other than inorganic/organic: gases, fumes, aerosols, chemicals, etc.
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12
Q

What is the name for the ILD conditions caused by stone dust, coal dust and asbestos fibres?

What is the brief pathophysiology?

A

Stone dust: silicosis
Coal dust: pneumoconiosis
Asbestos Fibres: asbestosis

  1. particles <7mm across reach terminal bronchioles and alveolar ducts
  2. Scavenged by macrophages
  3. Trigger release of mediators causing inflammation, tissue damage and scarring (fibrosis)
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13
Q

What could form in the lungs as a result of asbestos?

What cancers are you at risk of?

A

Pleural plaques, benign pleural effusion, diffuse pleural thickening

Mesothelioma, broncho-carcinoma

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14
Q

Which individuals are more at risk of asbestos exposure, how long can it take for symptoms of an asbestos-related condition to appear?

A

Carpenters, plumbers, electricians, painters, builders

10-40 years

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15
Q

What is hypersensitivity pneumonitis?

What can occur in the acute and chronic versions?

A

Extrinsic allergic alveolitis: inhalation of organic or inorganic antigens can trigger an immune response that results in alveolitis

Acute: produces an influenza like illness 4-9 hours after with dry cough and breathlessness on exertion

Chronic: long term exposure to antigens leads to dry cough and breathlessness for months-years

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16
Q

What are 2 common antigens that trigger hypersensitivity pneumonitis?

A
  1. Farmer’s lung; mouldy hay

2. Bird fancier’s lung; antigens from pigeons and budgies

17
Q

What are some treatment/management options for hypersensitivity pneumonitis?

A
  1. Bronchoalveolar lavage
  2. Surgical lung biopsy
  3. HRCT
  4. Avoidance and steroids
18
Q

What is sarcoidosis?

Which organs are commonly affected?

A

Multi-system granulomatous disorder of unknown cause but with genetic predisposition

Can affect any organ; but commonly lungs, skin and eyes

19
Q

What is the epidemiology of sarcoidosis?

A

Highest in young-middle aged, afro Caribbean, Asians and whites, female

20
Q

How does sarcoidosis initially present and what will a biopsy show?

A

Asymptomatic initially, a biopsy will show non-caseating granulomas

21
Q

List 3 reasons someone may acquire treatment-related ILD

A
  1. Certain drugs like methotrexate
  2. Radiotherapy
  3. Chemotherapy
22
Q

How might you recognize sarcoidosis on an X ray?

A

Bilateral hilar lymphadenopathy, pulmonary infiltration

23
Q

How would sarcoidosis appear on a lung function test?

A

Obstructive or restrictive

24
Q

Name 2 systemic CT diseases that can lead to ILD

A
  1. Rheumatoid arthritis

2. Scleroderma

25
Q

What is idiopathic interstitial lung disease?

What group of inflammatory disorders of the interstitium can it give rise to?

A

Inflammation of interstitial tissues with unknown cause

Can give rise to idiopathic interstitial pneumonias (all have differing clinical, histology features and prognosis)

26
Q

What is the most common Idiopathic interstitial pneumonia disorder? State a brief pathophysiology

A

IFP/UIP
Progressive inflammatory condition of lung
1. Increased number of activated alveolar macrophages
2. activated macrophages attract neutrophils and eosinophils which reactivate oxidant species and proteases
3. result is tissue destruction and fibrosis

27
Q

Which layer of the pleural seal has pain innervation?

A

The parietal pleura

28
Q

When would production of pleural fluid increase?

A
  1. Increase hydrostatic pressure in capillaries
  2. Increased permeability of capillaries
  3. Decreased oncotic pressure
29
Q

How is pleural fluid produced and absorbed?

A

Produced by filtration of capillaries and absorbed by lymphatic drainage

30
Q

List 3 causes of transudate

A

Heart failure, liver failure (hypoproteinemia), renal failure

31
Q

List 3 causes of exudate

A

Cancer, infection, CT disease

32
Q

What is the name of a primary pleural malignancy? How does it present and what commonly causes this?

A

Mesothelioma, commonly asbestos exposure and with pain and breathlessness

33
Q

What is Pectus carinatum and pectus excavatum?

What part of the breathing cycle will these affect?

A

Congenital chest wall diseases: pectus deformities

Pectus carinatum: protrusion of the anterior chest wall (pigeon chest)

Pectus excavatum: funnel chest (which can cause kyphosis, scoliosis and a muscular dystrophy)

More restrictive diseases and so affect inspiration

34
Q

How would you get a chest wall disease?

A

Congenital or acquired

35
Q

How would you acquire a chest wall disease?

A
  1. Trauma most common; can cause pneumothorax and lung collapse
  2. Anklyosing spondylitis: spinal arthritis
  3. Kyphosis and scoliosis