Interstitial Lung Disease Flashcards
What is interstitial lung disease (ILD)?
This is the generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner.
They are characterised by chronic inflammation and/or progressive interstitial fibrosis, and share a number of clinical and pathological features.
What is fibrosis?
Fibrosis involves the replacement of the normal elastic and functional lung tissue with scar tissue that is stiff and does not function effectively.
Give examples of causes of fibrosis
What are the 3 categories of ILD?
The ILDs can be broadly grouped into three categories:
- Those with a known cause
- Associated with systemic disorders
- Idiopathic
Give examples ILDs with known causes
- Occupational/environmental
- Asbestosis, berylliosis, silicosis and cotton worker’s lung (byssinosis)
- Drugs
- Nitrofurantoin, bleomycin, amiodarone, sulfasalazine and busulfan
- Hypersensitivity reactions
- Hypersensitivity pneumonitis
- Infections
- TB, fungi and viral
- Gastro-oesophageal reflux
Give examples of ILDs associated with systemic disorders
- Sarcoidosis
- Rheumatoid arthritis
- SLE, systemic sclerosis, mixed connective tissue disease and Sjögren’s syndrome
- Ulcerative colitis, renal tubular acidosis and autoimmune thyroid disease
Give examples of idiopathic ILDs
- Idiopathic pulmonary fibrosis
- Acute interstitial pneumonia (can be acute or subacute)
- Desquamative interstitial pneumonia (associated with smoking)
What drugs are linked to ILDs?
Methotrexate, nitrofurantoin, bleomycin, amiodarone, sulfasalazine and busulfan.
What are the signs of ILD?
- Bilateral fine end-inspiratory crepitations
- Dullness to percussion
- Finger clubbing
- Skin signs (e.g. Raynaud’s phenomenon in systemic sclerosis and erythema nodosum in sarcoidosis)
- Arthritis
Why may bilateral fine end-inspiratory crepitations be heard in ILD?
Bilateral fine end-inspiratory crepitations may be heard due to the sudden opening of small airways during inspiration, which were held closed during the previous expiration.
Why may there be dullness to percussion in ILD?
Dullness to percussion can be due to pleural effusion, which can occur in sarcoidosis and some connective tissue diseases.
What are the symptoms of ILD?
- Progressive exertional dyspnoea (usually presents slowly, over many weeks and months)
- Dry cough
- Connective tissue disease symptoms, such as arthralgia, difficulty swallowing and dry eyes
- General malaise and fatigue (due to underlying connective tissue disease or vasculitis)
Briefly describe what is shown on the CXR
AP chest radiograph showing air-space shadowing in the left upper zone. Although this appearance often represents infection, it is non-specific.
Differential diagnosis for this distribution of shadowing include lymphoma, alveolar cell carcinoma (both to be considered if not resolving in appearance on follow-up imaging), and haemorrhage.
What investigations should be ordered for ILD?
- FBC
- ESR and CRP
- Autoimmune antibodies
- CXR
- High-resolution CT (HRCT)
- Spirometry
- Bronchoalveolar lavage (BAL)
- Trans-bronchial biopsy/surgical lung biopsy
Why investigate FBC?
May show anaemia of chronic disease.
Why investigate ESR and CRP?
May be elevated.