Idiopathic pulmonary fibrosis

Question 1

what is IPF?

Answer 1

• it is an interstitial lung disease
  which means it affects the lung tissue
  causing inflammation and fibrosis (scar tissue)
• idiopathic bc there is no clear cause

Question 2

which age range and gender is IPF typically seen in?

Answer 2

men

50-70 years old

Question 3

How does IPF present?

Answer 3

1. progressive exertional dyspnoea
2. bibasal fine end-inspiratory crepitations on auscultation
3. dry cough
4. clubbing

Question 4

what investigations are done for IPF and what are the results?

which investigation is best for diagnosing?

Answer 4

1. spirometry - restrictive picture FEV1 normal/decreased, FVC- significantly decreased. FEV1/FVC increased (>0.7)
2. Ground-glass appearance, progressing to honeycombing on CXR and CT (high resolution CT for diagnosing)
3. impaired gas exchange (reduced TLCO)
4. ANA and Rh factor can be positive but does not imply underlying connective tissue disease is cause

Question 5

what is the management for IPF?

Answer 5

1. pulmonary rehabilitation
2. perfenidone (anti-fibrotic) has some evidence of benefit in some patients
3. supplemental oxygen
4. pneumococcal and flu vaccine
5. eventually lung transplant

Question 6

what is the prognosis of IPF?

Answer 6

poor, 2-4 years

Question 7

What are other examples of interstitial lung disease other than idiopathic pulmonary fibrosis?

Answer 7

1. Drug induced pulmonary fibrosis
2. Secondary pulmonary fibrosis
3. hypersensitivity pneumonitis/ EAA
4. Cryptogenic organising pneumonia
5. Asbestosis

Question 8

What drugs cause drug induced pulmonary fibrosis?

Answer 8

1. Amiodarone
2. Phenytoin
3. Methotrexate
4. abxs such as Nitrofurantoin

Question 9

What conditions can cause secondary pulmonary fibrosis?

Answer 9

1. Rheumatoid arthritis
2. Alpha-1 Antitrypsin deficiency
3. SLE
4. Systemic Sclerosis

Question 10

What is cryptogenic organising pneumonia?

How does it present ?

How do you diagnose it definitively?

How do you treat it?

Answer 10

• involves a focal area of inflammation of the lung tissue
• this can be idiopathic, triggered by infection, drugs, inflammatory disorder
• presents like pneumonia - cough, sob, fever, lethargy.
• CXR also presents with consolidation
• diagnosed definitively using biopsy. can take some time due to similarity to pneumonia
• Treat with systemic corticosteroids

Question 11

What is Asbestosis?

What two features does asbestos have?

What can Asbestos inhalation cause?

What are patients with conditions related to asbestos eligible for and what must you do if they die?

Answer 11

1. lung disease due to asbestos inhalation
2. fibrogenic (causes fibrosis), oncogenic
3. lung fibrosis, mesothelioma, adenocarcinoma, pleural thickening and pleural plaques
4. pts eligible for compensation. all must be referred to coroner