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Respiratory Physiology, Medicine and Critical Care > Restrictive lung disease - Idiopathic pulmonary fibrosis > Flashcards

Restrictive lung disease - Idiopathic pulmonary fibrosis Flashcards

1
Q

What is idiopathic pulmonary fibrosis?

A

Also known as usual interstitial pneumonia (UIP) and was previously known as cryptogenic fibrosing alveolitis (CFA).

It is described as a chronic irreversible and ultimately fatal disease characterized by a progressive decline in lung function.

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2
Q

What is the pathogenesis of IPF?

A

Multiple injuries to the alveolar cells cause them to secrete growth factors that recruit fibroblasts in a fibrotic environment. These differentiate into myofibroblasts under the influence of TNF-beta, synthesize collagen and aggregate in fibrotic foci.

In essence, this is a remodelling response gone wrong, and results in heterogeneous irreversible fibrosis

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3
Q

What are the histological features of UIP?

A
  • Patchy interstitial chronic inflammation
  • Type II pneumocyte hyperplasia
  • Proliferating fibroblastic foci
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4
Q

What are symptoms of UIP?

A
  • Dry cough
  • Exertional dyspnoea
  • Malaise
  • Weight loss
  • Arthralgia
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5
Q

What are signs of UIP?

A
  • Cyanosis
  • Finger clubbing
  • Fine-end inspiratory crepitations
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6
Q

How would you investigate someone who you suspected had UIP?

A
  • Bloods - ABG, CRP, Immunoglobulin, ANA, RF
  • CXR
  • HRCT
  • Spirometry and gas transfer
  • BA lavage
  • Lung biopasy
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7
Q

What might you see on ABG in someone with UIP?

A
  • Type I respiratory failure - Decreased PaO2
  • Type II respiratory failure in severe cases - Increased PaCO2
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8
Q

What happens to the CRP in someone with UIP?

A

Increased CRP

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9
Q

What inflammatory markers can be increased in UIP?

A
  • ANA
  • RF
  • Immunoglobulins
  • CRP
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10
Q

What might you see on CXR in someone with UIP?

A
  • Decreased lung volume
  • Bilateral lower zone reticulonodular shadows
  • Honeycomb lung - advanced disease
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11
Q

What might you see on CT scanning in someone with UIP?

A
  • Honeycombing
  • Reticular opacities: subpleural lung - associated with honeycombing and traction bronchiectasis
  • Ground-glass opacities
  • Lung architectural distortion
  • Lobar volume loss (predominantly lower lobes)
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12
Q

What will you see on spirometry and gas transfer in someone with UIP?

A

Restrictive lung pattern + reduced gas transfer

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13
Q

What might you see on BA lavage in someone with UIP?

A

Increased lymphocytes/neutrophils/eosinophils

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14
Q

What are complications of UIP?

A
  • Respiratory failure
  • Lung cancer risk
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15
Q

What is the differential diagnosis for someone with features of UIP?

A
  • Hypersensitivity pneumonitis
  • Bronchiectasis
  • Chronic left heart failure
  • Sarcoidosis
  • Industrial lung disease
  • Lymphangitis carcinomatosa
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16
Q

What are the functional effects of restrictive lung disease?

A
  • Reduced Lung Compliance - due to lung stiffness
  • Low FEV1, Low FVC
  • FEV1/FVC normal ratio
  • Reduced Gas Transfer (Tco or Kco)
  • Ventilation/Perfusion Imbalance
17
Q

What is honeycombing of the lung?

A

Honeycomb lung refers to the fibrotic cystic changes to the lungs in end stage interstitial disease. The formation of fibrotic interstitium leads to reduced gas diffusion, in particular oxygen. However, as CO2 is much more soluble than oxygen, its diffusion is not affected significantly, and CO2 levels do not change. This means that any type of respiratory failure associated with restrictive lung diseases is almost always TYPE I RESPIRATORY FAILURE.

18
Q

Why does PaO2 drop on exertion in interstitial lung disease?

A

Equilibration of gases normally takes 0.25 secs for both oxygen and CO2. The amount of time that RBCs spend in the pulmonary capillaries is approximately 0.75s. In restrictive lung disease, the gas equilibration time is closer to 0.75s, meaning that less gas can transfer across the membrane in the time that the RBCs are transitioning through the capillaries.

This results in near normal resting PaO2, but serious drops in PaO2 when exercising (this can be treated by increasing the fraction of inspired air which is oxygen, which increases the pressure gradient between alveoli and capillaries, resulting in faster diffusion).

19
Q

What is the survival rate of someone who is diagnosed with UIP?

A

3 years

20
Q

How would you manage someone with UIP?

A

Best supportive care

  • O2
  • Pulmonary rehabilitation
  • Opiates
  • Palliative care
21
Q

What are secondary causes of UIP?

A
  • Connective tissue diseases - esp scleroderma and rheumatoid disease
  • Drug reaction
  • Post infection
  • Industrial exposure - asbestos
22
Q

What mnemonic can you use to remember some of the aetiologies of pulmonary fibrosis?

A

BREAST RAC

  • Bleomycin
  • Radiation
  • Extrinsic Allergic alveolitis
  • Ankylosing spondylitis
  • Sarcoidosis
  • Tuberculosis
  • Rheumatoid arthritis
  • Asbestosis
  • Cryptogenic fibrosing alveolitis
23
Q

If you suspected IPF, what are the primary investigations you would consider doing?

A
  • PFTs
  • CXR
  • HRCT
24
Q

What are causes of upper zone fibrosis?

A

CARTEX

  • Coal
  • ANkylosing spondylitis
  • Radiation
  • TB
  • Extrinsic allergic alveolitis
25
Q

What are causes of lower zone fibrosis?

A

CAID

  • Connective tissue disease - scleroderma, RA
  • Asbestosis
  • IPF
  • Drugs

Respiratory Physiology, Medicine and Critical Care (42 decks)

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