Interstitial Lung Disease Flashcards

1
Q

What is interstitial lung disease (ILD)?

A

This is the generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner.

They are characterised by chronic inflammation and/or progressive interstitial fibrosis, and share a number of clinical and pathological features.

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2
Q

What is fibrosis?

A

Fibrosis involves the replacement of the normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively.

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3
Q

Give examples of causes of fibrosis

A
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4
Q

What are the 3 categories of ILD?

A

The ILDs can be broadly grouped into three categories:

  • Those with a known cause
  • Associated with systemic disorders
  • Idiopathic
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5
Q

Give examples ILDs with known causes

A
  • Occupational/environmental
    • Asbestosis, berylliosis, silicosis and cotton worker’s lung (byssinosis)
  • Drugs
    • Nitrofurantoin, bleomycin, amiodarone, sulfasalazine and busulfan
  • Hypersensitivity reactions
    • Hypersensitivity pneumonitis
  • Infections
    • TB, fungi and viral
  • Gastro-oesophageal reflux
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6
Q

Give examples of ILDs associated with systemic disorders

A
  • Sarcoidosis
  • Rheumatoid arthritis
  • SLE, systemic sclerosis, mixed connective tissue disease and Sjögren’s syndrome
  • Ulcerative colitis, renal tubular acidosis and autoimmune thyroid disease
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7
Q

Give examples of idiopathic ILDs

A
  • Idiopathic pulmonary fibrosis
  • Acute interstitial pneumonia (can be acute or subacute)
  • Desquamative interstitial pneumonia (associated with smoking)
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8
Q

What drugs are linked to ILDs?

A

Methotrexate, nitrofurantoin, bleomycin, amiodarone, sulfasalazine and busulfan.

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9
Q

What are the signs of ILD?

A
  • Bilateral fine end-inspiratory crepitations
  • Dullness to percussion
  • Finger clubbing
  • Skin signs (e.g. Raynaud’s phenomenon in systemic sclerosis and erythema nodosum in sarcoidosis)
  • Arthritis
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10
Q

Why may bilateral fine end-inspiratory crepitations be heard in ILD?

A

Bilateral fine end-inspiratory crepitations may be heard due to the sudden opening of small airways during inspiration, which were held closed during the previous expiration.

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11
Q

Why may there be dullness to percussion in ILD?

A

Dullness to percussion can be due to pleural effusion, which can occur in sarcoidosis and some connective tissue diseases.

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12
Q

What are the symptoms of ILD?

A
  • Progressive exertional dyspnoea (usually presents slowly, over many weeks and months)
  • Dry cough
  • Connective tissue disease symptoms, such as arthralgia, difficulty swallowing and dry eyes
  • General malaise and fatigue (due to underlying connective tissue disease or vasculitis)
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13
Q

Briefly describe what is shown on the CXR

A

AP chest radiograph showing air-space shadowing in the left upper zone. Although this appearance often represents infection, it is non-specific.

Differential diagnosis for this distribution of shadowing include lymphoma, alveolar cell carcinoma (both to be considered if not resolving in appearance on follow-up imaging), and haemorrhage.

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14
Q

What investigations should be ordered for ILD?

A
  • FBC
  • ESR and CRP
  • Autoimmune antibodies
  • CXR
  • High-resolution CT (HRCT)
  • Spirometry
  • Bronchoalveolar lavage (BAL)
  • Trans-bronchial biopsy/surgical lung biopsy
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15
Q

Why investigate FBC?

A

May show anaemia of chronic disease.

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16
Q

Why investigate ESR and CRP?

A

May be elevated.

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17
Q

Why investigate autoimmune antibodies?

A

Anti-CCP suggests rheumatoid arthritis, ANA suggests SLE.

Autoimmune antibodies may also be raised in idiopathic pulmonary fibrosis.

18
Q

Why investigate using CXR?

A

Typically shows reticular (fine) opacities. The location of fibrosis may suggest particular causes.

19
Q

What are the causes of upper zone fibrosis?

A
20
Q

What are the causes of lower zone fibrosis?

A
21
Q

Why investigate using high-resolution CT (HRCT)?

A

HRCT is preferred to volume CT as it provides higher quality images of the lung parenchyma, which is the area affected in ILD. Some ILDs, especially idiopathic pulmonary fibrosis, show a “usual interstitial pneumonia” pattern, which includes:

  • Honeycombing (clusters of cystic airspaces)
  • Traction bronchiectasis (dilated airways, which are pulled apart by areas of surrounding fibrosis)
  • Reticular opacities (thickening of the lung interstitium)
22
Q

What is shown on the CXR?

A

Chest x-ray showing lower zone fibrosis.

23
Q

Why investigate using spirometry?

A

Restrictive pulmonary spirometry.

24
Q

Why investigate using bronchoalveolar lavage (BAL)?

A

A bronchoscope (small camera) is inserted into the airways, and sterile saline is injected through the bronchoscope into the airways. The saline is then collected via suction and analysed. This helps to diagnose the underlying cause of the ILD, and to also exclude infection or malignancy.

25
Q

Why investigate using trans-bronchial biopsy/surgical lung biopsy?

A

A small sample of lung is collected and then analysed by a pathologist. This may be needed if previous tests are inconclusive, or if a tissue sample is needed to make the diagnosis.

26
Q

What are the pathological findings of ILD?

A

Fibrosis and remodelling of the interstitium; chronic inflammation; hyperplasia of type II epithelial cells or type II pneumocytes.

27
Q

Medical management varies for each type of ILD. What are general principles of management?

A

Generally there is a poor prognosis and limited management options in interstitial lung disease as the damage is irreversible. Generally the treatment is supportive and where possible to prevent further progression of the disease. Options are:

  • Remove or treat the underlying cause
  • Home oxygen where they are hypoxic at rest
  • Stop smoking
  • Physiotherapy and pulmonary rehabilitation
  • Pneumococcal and flu vaccine
  • Advanced care planning and palliative care where appropriate
  • Lung transplant is an option but the risks and benefits need careful consideration
28
Q

What are the indications for long-term oxygen therapy (LTOT)?

A

Some patients with ILD may benefit from LTOT. Indications for LTOT are one of the following:

  • SpO2 <88%
  • PaO2 <7.3kPa

LTOT is contraindicated in current smokers due to the risk of burns.

29
Q

When may surgical management be considered for ILD?

A

For patients with severely impacted quality-of-life, despite optimum medical management.

30
Q

What are the complications of ILD?

A
  • Disease related complications:
    • Respiratory failure: due to failure of gas exchange in the lungs
    • Pulmonary hypertension: due to chronic hypoxic pulmonary vasoconstriction
    • Anxiety and depression: due to impaired quality-of-life
  • Treatment related complications:
    • Due to long-term steroid use: osteoporosis, hypertension and Cushing’s syndrome
    • Due to antifibrotics: diarrhoea, nausea and anorexia
31
Q

What is the medical management of ILD due to idiopathic lung fibrosis, sarcoidosis, connective tissue disease and extrinsic allergic alveolitis?

A

Idiopathic pulmonary fibrosis: antifibrotics (nintedanib or pirfenidone)

Sarcoidosis: corticosteroids (prednisolone)

Connective tissue disease: corticosteroids and steroid-sparing agents (azathioprine or mycophenolate)

Extrinsic allergic alveolitis: corticosteroids

32
Q

What differentials should be considered for ILD?

A
  1. COPD
  2. Asthma
  3. Congestive heart failure
  4. Lung cancer
  5. Bronchiectasis
33
Q

How does ILD and COPD differ?

A

Differentiating signs and symptoms:

  • Cough is more productive of sputum

Differentiating investigations:

  • Lung function tests: obstructive pattern (FEV1/FVC < 70%)
  • Examination and chest X-Ray: chest hyperinflation
34
Q

How does ILD and asthma differ?

A

Differentiating signs and symptoms:

  • Diurnal variation in symptoms and peak flow
  • History of atopy

Differentiating investigations:

  • Lung function tests: bronchodilator reversibility
35
Q

How does ILD and congestive cardiac failure differ?

A

Differentiating signs and symptoms:

  • Orthopnoea (dyspnoea while lying down)
  • Paroxysmal nocturnal dyspnoea (sudden dyspnoea which wakes the patient up from sleep)

Differentiating investigations:

  • Blood tests: elevated BNP
  • Echocardiogram: reduced ejection fraction
36
Q

How does ILD and lung cancer differ?

A

Differentiating signs and symptoms:

  • Significant weight loss
  • Haemoptysis

Differentiating investigations:

  • Imaging: visible tumour
37
Q

How does ILD and bronchiectasis differ?

A

Differentiating signs and symptoms:

  • Cough is more productive of sputum
  • More frequent lower respiratory tract infections, often starting in childhood

Differentiating investigations:

  • Lung function tests: obstructive pattern (FEV1/FVC < 70%)
38
Q

How long is the life expectancy following diagnosis?

A

2-5 years.

39
Q

What medication can be prescribed to slow down progression?

A

Pirfenidone and nintedanib.

40
Q

What fingernail changes are observed in ILD?

A

Clubbing.