Idiopathic pulmonary fibrosis

Question 1

Features

Answer 1

Also known as:

• cryptogenic fibrosing alveolitis
• or idiopathic fibrosing interstitial pneumonia

is the most common diagnosis among pts presenting with interstitial lung disease.

Question 2

Diagnosis

Answer 2

Pts usually present in the 5th–7th decade

• with the clinical features as outlined under interstitial lung diseases.

CXR abnormalities are variable but include:

• bilateral diffuse nodular or
• reticulonodular shadowing favouring the lung bases.

Open lung biopsy may be needed for diagnosis and staging.

Question 3

The usual treatment is

Answer 3

high doses of oral corticosteroids with azathioprine.

Prognosis is poor.

Question 4

Hypersensitive pneumonitis (extrinsic allergic alveolitis)

Answer 4

characterised by a widespread diffuse inflammatory reaction in both the small airways of the lung and alveoli,

• due to the inhalation of allergens,
• which are usually spores of micro-organisms such as
• Thermophilic actinomycetes in ‘farmer’s lung’ or (more commonly) avian proteins from droppings or feathers in ‘bird fancier’s lung’.

Management is based on prevention, namely avoiding exposure to allergens or wearing protective fine mesh masks.

Prednisolone can be used (with caution) to control acute symptoms.

It should be pointed out that this allergic disease is different to the infection psittacosis.