Interstitial Lung Disease Flashcards
What are interstitial lung diseases? What is fibrosis?
Include many conditions that cause inflammation and fibrosis of the lung tissue.
Fibrosis involves the replacement of elastic and functional lung tissue with non-functional scar tissue.
List the conditions that come under interstitial lung diseases.
- idiopathic pulmonary fibrosis
- secondary pulmonary fibrosis
- hypersensitivity pneumonitis
- cryptogenic organising pneumonia
- asbestosis
What are the typical presentations of patients with ILD?
- SOB on exertion
- Dry cough
- Fatigue
What are the characteristic findings of idiopathic pulmonary fibrosis? (Very imp)
- bibasal fine end-inspiratory crackles
- finger clubbing
How do you diagnose ILD?
- Clinical features
- HRCT of the thorax (shows a typical ground glass appearance)
- Spirometry (may be normal or show a restrictive pattern)
Note: If in doubt, other investigations you could do are lung biopsy and bronchoalveolar lavage.
What is the general management for ILD, and what is its prognosis like?
- remove or treat the underlying cause
- home oxygen where there is hypoxia
- stop smoking
- physio/ pulmonary rehab
- pneumococcal and flu vaccine
- advanced care planning and palliative care
- lung transplant
What happens in Idiopathic pulmonary fibrosis? Who does it usually affect, and what is its prognosis like?
- progressive pulmonary fibrosis with no apparent cause
- presents with insidious onset of SOB and dry cough for more than 3 months
- Usually affects adults over the age of 50 years.
- the prognosis if poor, with a 2-5 years life expectancy from diagnosis
Which drugs and conditions can cause secondary pulmonary fibrosis?
Drugs:
- Amiodarone
- Cyclophosphamide
- Methotrexate
- Nitrofurantoin
Conditions:
- alpha-1 trypsin deficiency
- rhematoid arthritis
- SLE
- Systemic sclerosis
- sarcoidosis
What happens in hypersensitivity pneumonitis?
Inhalation of allergens in patients sensitised to that allergen causes an immune response leading to inflammation and damage to the lung tissue.
How do you investigate hypersensitivity pneumonitis?
Bronchoalveolar lavage is performed during a bronchoscopy procedure.
Raised lymphocytes are suggestive of this condition.
What are some causes of hypersensitivity pneumonitis?
- bird-fancier’s lung- reaction to bird droppings
- Farmer’s lung- mouldy spores in hay
- Mushroom worker’s lung- mushroom antigens
- malt worker’s lung- mould on barley
What is cryptogenic organizing pneumonia? And why is it hard to diagnose it?
It involves a focal area of inflammation of the lung tissue. It can be idiopathic or triggered by infection, inflammatory disorders, medications, radiation, environmental toxins, or allergens.
Presentation is similar to infectious pneumonia, with shortness of breath, cough, fever and lethargy. Inspiratory crackles may be heard on auscultation.
Chest x-ray findings are also similar to pneumonia, with a focal consolidation.
What is a definitive diagnostic tool for Cryptogenic organising pneumonia? What is its treatment?
diagnosis- Lung biopsy
treatment- systemic corticosteroids
What is asbestosis? What problems does it cause?
Lung fibrosis related to asbestos exposure. 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.
- lung fibrosis
- pleural thickening
- adenocarcinoma
- mesothelioma
