Fibrotic/Intestitinal Lung Disease

Question 1

What

Answer 1

Interstitial lung disease is an umbrella term to describe conditions that affect the lung parenchyma (the lung tissue) causing inflammation and fibrosis. Fibrosis involves the replacement of the normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively.

Question 2

Diagnosis

Answer 2

Diagnosis of interstitial lung disease requires a combination of clinical features and high resolution CT scan of the thorax. HRCT shows a “ground glass” appearance with interstitial lung disease. When diagnosis is unclear lung biopsy can be used to take samples of the lung tissue and confirm the diagnosis on histology.

Question 3

Treat

Answer 3

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

Question 4

Idiopathic Pul fibrosis

Answer 4

This is a condition where there is progressive pulmonary fibrosis with no clear cause. It presents with an insidious onset of shortness of breath and dry cough over more than 3 months. It usually affects adults over 50 years old. Examination can show bibasal fine inspiratory crackles and finger clubbing. Prognosis is poor with a life expectancy of 2-5 years from diagnosis.

Two medications are licensed that can slow the progression of the disease:

Pirfenidone is an antifibrotic and anti-inflammatory
Nintedanib is a monoclonal antibody targeting tyrosine kinase

Question 5

Drug Induced Pul Fibrosis

Answer 5

There are several drugs that can cause pulmonary fibrosis. Key medication that are worth remembering are:

Amiodarone
Cyclophosphamide
Methotrexate
Nitrofurantoin

Question 6

Secondary Pul Fibrosis

Answer 6

Pulmonary fibrosis can occur secondary to other conditions:

Alpha-1 antitripsin deficiency
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Systemic sclerosis

Question 7

Hypersensitivity Pneumonitis

Answer 7

Hypersensitivity pneumonitis is a type III hypersensitivity reaction to an environmental allergen that causes parenchymal inflammation and destruction in people that are sensitive to that allergen. Bronchoalveolar lavage involves collecting cells from the airways during bronchoscopy by washing the airways with fluid then collecting that fluid for testing. This shows raised lymphocytes and mast cells in hypersensitivity pneumonitis.

Management is by removing the allergen, giving oxygen where necessary and steroids.

Examples of specific causes:

Bird-fanciers lung is a reaction to bird droppings
Farmers lung is a reaction to mouldy spores in hay
Mushroom workers’ lung is a reaction to specific mushroom antigens
Malt workers lung is a reaction to mould on barley

Question 8

Asbestosis

Answer 8

Asbestosis is lung fibrosis related to the inhalation of asbestos. Asbestos is fibrogenic, meaning it causes lung fibrosis. It is also oncogenic, meaning it causes cancer. The effects of asbestos usually take several decades to develop. Asbestos inhalation causes several problems:

Lung fibrosis
Pleural thickening and pleural plaques
Adenocarcinoma
Mesothelioma
Suffers are eligible for compensation if they develop asbestos related health conditions (except isolated pleural plaques). All patients that die with known exposure to asbestos need to be referred to the coroners.