INTERSTITIAL LUNG DISEASE

Question 1

Define interstitial lung disease.

Answer 1

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

Question 2

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

Answer 2

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

Question 3

What is the most common ILD?

Answer 3

Usual interstitial pneumonitis (UIP)

Question 4

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

Answer 4

Hypersensitivity to a number of possible allergens. Eg birds.

Question 5

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

Answer 5

A restrictive pattern.

Question 6

What is a restrictive pattern in a lung function test?

Answer 6

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

Question 7

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

Answer 7

Yes. They will both be reduced.

Question 8

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

Answer 8

Asbestos
Crystalline Silica
Beryllium

Question 9

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

Answer 9

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

Question 10

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

Answer 10

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

Question 11

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

Answer 11

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

Question 12

Name 4 interstitial lung diseases that are considered idiopathic.

Answer 12

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

Question 13

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

Answer 13

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

Question 14

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

Answer 14

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

Question 15

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

Answer 15

Chest x-ray
Chest CT
Lung function test

Question 16

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

Answer 16

Widespread or local areas of haziness

Question 17

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

Answer 17

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.

Question 18

Can idiopathic pulmonary fibrosis be reversed and cured?

Answer 18

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

Question 19

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

Answer 19

Type 1

Question 20

What are the possible complications of idiopathic pulmonary fibrosis?

Answer 20

Type 1 respiratory failure
Heart failure
Infection
Lung cancer

Question 21

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

Answer 21

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.