INTERSTITIAL LUNG DISEASE Flashcards

1
Q

Define interstitial lung disease.

A

Chronic inflammation leading to fibrosis of the lung parenchyma. This reduces compliance of the lung tissue and hence leads to difficulty breathing.

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2
Q

In ILD, is it the alveoli or the airways that are affected?

A

Alveoli. The disease may involve the alveoli themselves, the interstitial space or the capillaries.

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3
Q

What is the most common ILD?

A

Usual interstitial pneumonitis (UIP)

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4
Q

What are most acute episodes of parenchymal inflammation the result of?

A

Hypersensitivity to a number of possible allergens. Eg birds.

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5
Q

What pattern will be seen in lung function tests with someone with ILD?

A

A restrictive pattern.

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6
Q

What is a restrictive pattern in a lung function test?

A

Normal or even increased FEV1/FVC ration but a reduced FEV1 and FVC.

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7
Q

Will the diffusing capacity (DLCO) and the (KCO) be affected in a patient with ILD?

A

Yes. They will both be reduced.

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8
Q

What inhaled substances often associated with a profession may lead to someone developing ILD? (Name 3)

A

Asbestos
Crystalline Silica
Beryllium

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9
Q

Which classes of drug can lead to a patient developing ILD? Give an example of a specific drug for each class. (Name 5 classes)

A

Antibiotics - Nitrofurantoin
Chemotherapeutic drugs - Methotraxate, Bleomycin, Busulfan
Anti-arrythmics - Amiodarone
Some drugs used to treat rheumatoid arthritis - Sulfasalazine
Statins - Simvastatin (more than atorvastatin)

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10
Q

Which connective tissue disorders are associated with ILD? (Name at least 4, there are 6 given)

A
Rheumatoid arthritis
Systemic Lupus Erythematosus (SLE)
Sjorgen's syndrome
Systemic sclerosis
Polymyositosis
Dermatomyositosis
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11
Q

Which infections are recognised as being risk factors for developing ILD? (Name all 5)

A
Tuberculosis
Chlamydia Trachomatis
Respiratory syncytial virus (RSV)
Atypical pneumonias
Pneumocystic pneumonias
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12
Q

Name 4 interstitial lung diseases that are considered idiopathic.

A

Hamman-Rich syndrome (also called Acute Interstitial Pneumonitis)
Idiopathic Pulmonary Fibrosis
Cryptogenic Organising Pneumonitis
Lymphocytic Interstitial Pneumonitis
Sarcoidosis - not classically considered an ILD but the presenting picture is so similar

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13
Q

How would a patient with suspected ILD normally present? (Name 2 symptoms)

A

Dyspnoea on exertion
Paroxysmal (suddenly getting worse) non-productive cough
Weight loss
Malaise

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14
Q

On examination what might you find in a patient with suspected ILD? (Name 2 signs)

A

Cyanosis
Abnormal breath sounds - fine end inspiratory crepitations
Clubbing
O2 sats may also be low

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15
Q

What investigations would be needed to confirm a diagnosis of ILD?

A

Chest x-ray
Chest CT
Lung function test

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16
Q

What are you likely to see on a chest x-ray of someone with ILD?

A

Widespread or local areas of haziness

17
Q

What are you likely to see on a chest CT of someone with ILD?

A

A CT could reveal areas of ground glass or honeycombing. This might help to pinpoint a more specific diagnosis based on the pattern of disease.

18
Q

Can idiopathic pulmonary fibrosis be reversed and cured?

A

No. Treatment is focused on reducing symptoms, stopping disease progression, preventing acute exacerbations and prolonging survival.

19
Q

Is someone with idiopathic pulmonary fibrosis more likely to develop type 1 or type 2 respiratory failure?

A

Type 1

20
Q

What are the possible complications of idiopathic pulmonary fibrosis?

A

Type 1 respiratory failure
Heart failure
Infection
Lung cancer

21
Q

What are the treatment options for someone with idiopathic pulmonary fibrosis (IPF)? (Name 5 things that can be done to manage the disease)

A

Pirfenidone - an anti-inflammatory, anti-oxidant and anti-fibrotic agent. It prevents fibroblast proliferation. Some evidence to suggest is may halt disease progression in mild cases.

Long term oxygen therapy (LTOT)

Opiates - considered for those with severe IPF (often part of palliative care)

Vaccinations - preventing further infections is the best way to prevent disease progression.

Smoking cessation

Pulmonary physiotherapy and exercise

Single lung transplant - in those patients that are fit enough to survive major operation.