Interstitial lung disease Flashcards

1
Q

What is ILD?

A

Interstitial lung disease is an umbrella term to describe conditions that affect the lungparenchyma (the lung tissue) causinginflammation andfibrosis.

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2
Q

What are the different types?

A

200 different types
- Usual Interstitial Pneumonia (UIP)
- Non-specific Interstitial Pneumonia (NSIP)
- Extrinsic Allergic Alveolitis
- Sarcoidosis
- Idiopathic pulmonary fibrosis
- Several other conditions

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3
Q

What happens physiologically to the lung tissue?

A
  • alveolar capillary membrane is thickened
  • Increased collagen in alveloar walls- decreased compliance and increased elastic recoil
  • increased diffusion distance for O2 and CO2
  • Impaired gas exchange
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4
Q

Causes: specific exposure

A
  • Asbestos
  • Drugs/medications
  • Mouldy Hay- Farmer’s lung (EAA)
  • Dust- Silicosis
  • Coal- pneumoconiosis
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5
Q

What drugs/medication can cause ILD?

A
  • radiation
    • Chemotherapy
    • Amiodarone
    • Bleomycin
    • Methotrexate
    • Nitrofurantoin
    • Penicillamine
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6
Q

Causes: autoimmune?

A
  • Sarcoidosis
  • Rheumatoid arthritis
  • SLE
  • Schleroderma
  • Sjorgen’s
  • Polymyositis
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7
Q

Causes: unknown injury?

A

Fibrosing alveolitis- cryptogenic fibrosing alveolitis or idiopathic pul fibrosis (CFA/IPF)

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8
Q

Symptoms?

A
  • Dry cough
  • Dyspnoea on exertion
  • Fatigue
  • Typical gradual progressive Sx
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9
Q

Signs?

A
  • decreased lung expansion on palpation
  • Bi-basal end inspiratory crackles- “Velcro crackles”
  • Finger clubbing
  • Small pleural effusions
  • Cyanosis
  • Hypoxaemia
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10
Q

General investgations?

A
  • Detailed history important
  • Clinical examination
  • Spirometry
    • Typically restrictive lung diseases on PFT’s
      • FEV1/FVC ratio normal >0.7
      • however FVC lower than normal
  • CXR
  • Diagnosis: CT of the thorax
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11
Q

How to investigate the cause of the fibrosis?

A
  • bronchoalveolar lavage or transbronchial biopsyand/or
  • surgical lung biopsy, with the agreement of the thoracic surgeon.
    ANA – connective tissue disease OR SLE
    ENA – connective tissue disease
    Rh F – Rheumatoid Arthritis
    ANCA – Vasculitis
    Anti-GBM – Pulmonary Renal disease
    ACE – Sarcoidosis
    Ig G to serum precipitins e.g. pigeon, budgie-Extrinsic
    Allergic Alveolitis
    HIV
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12
Q

How to manage ILD?

A
  • Occupational exposure – remove
  • Environmental exposure – remove (e.g. pet birds)
  • Drug associated – avoid
  • Stop smoking
  • MDT approach – maybe suitable for specialist
    medications such as Pirfenidone to slow progression
  • Treatment of infective exacerbations
  • Oxygen if respiratory failure
  • Palliative care
  • Transplantation
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13
Q

Usual Interstitial pneumonia (UIP) what findings are there ?

A

Commonest cause of
- clubbing, reduced chest expansion
- Auscultation – fine inspiratory crepitations (like pulling Velcro slowly) – usually best heard basal / axillary areas
- Cardiovascular – may be features of pulmonary hypertension

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14
Q

What can cause Extrinsic Allergic Alveolitis?

A
  • Bird-fanciers lungis a reaction to bird droppings
  • Farmers lungis a reaction to mouldy spores in hay
  • Mushroom workers’ lungis a reaction to specific mushroom antigens
  • Malt workers lungis a reaction to mould on barley
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15
Q

Extrinsic Allergic Alveolitis: what is it known as and what type of reaction is it?

A

Also known as Hypersensitivity Pneumonitis

  • type III hypersensitivity reaction
  • Inhalation of organic antigen to which the individual has been sensitised
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16
Q

Extrinsic Allergic Alveolitis: 2 types and explain how manage ?

A
  • ACUTE – short period from exposure, 4-8 hrs. Usually reversible: spontaneously settle 1-3 days. Can reoccur.
  • CHRONIC – chronic exposure (months – years). Less reversible.
17
Q

Extrinsic Allergic Alveolitis: Investigations

A

Same as for ILD
Bronchoalveolar lavage involves collecting cells from the airways duringbronchoscopy by washing the airways with fluid then collecting that fluid for testing. Findings in hypersensitivity pneumonitis:

  • raised lymphocytes
  • mast cells
18
Q

Extrinsic Allergic Alveolitis: management

A
  • removing the allergen
  • oxygen where necessary
  • steroids.
19
Q

Sarcoidosis: What is it ?

A

Multisystem inflammatory condition of unknown cause
Immunological response

20
Q

Sarcoidosis: on histology?

A

Non-caseating granulomas

21
Q

Sarcoidosis: What does it commonly affect?

A

Commonly involves Resp system BUT can affect nearly all organs

22
Q

Sarcoidosis: investigations

A
  • PFTs: (obstructive until) fibrosis
  • CXR: 4 stages
  • Bloods: renal function, ACE, Calcium
  • Urinary Calcium
  • Cardiac involvement: ECG, 24 tape, ECHO, cardiac MRI
  • CT/MRI head: headaches – Neuro sarcoid