A restrictive lung disease characterised by accumulation of excess fibrous connective tissue in the lung parenchyma, causing reduced lung compliance and oxygen diffusion capacity. This leads to progressive hypoxaemia and shortness of breath.
Causes
Clinical features
Symptoms
- Chronic progressive shortness of breath
- Dry cough
Signs of pulmonary fibrosis
- Tachypnoea
- Reduced expansion
- Fine end-inspiratory crepitations
Signs of cause
- Clubbing (idiopathic pulmonary fibrosis)
- Hand deformity (RA)
- Telangiectasia, sclerodactyly and microstomia (systemic sclerosis)
- Butterfly rash (SLE)
- Lupus pernio (sarcoidosis)
- Radiation burns
- Kyphosis (ankylosing spondylitis)

Telangiectasia

Rheumatoid arthritis

Clubbing
Investigations
- Chest X-ray: reticulonodular shadowing
- High-resolution CT chest: honeycombing cysts, reticular septal thickening, ground glass changes (indicates inflammatory process which is often steroid responsive)
- Spirometry: restrictive defect with reduced diffusion capacity
- Blood tests: for likely causes, e.g. antinuclear antibody, rheumatoid factor, anti-centromere antibody
- Lung biopsy
Management
NB: fibrosis is permanent and there is no cure.
- Corticosteroids may help in acute exacerbationsÂ
- Antifibrotics (pirfenidone/nintedanib) may be used in idiopathic pulmonary fibrosis
- Treat/withdraw cause if possible (may include corticosteroids and immunosuppressants)
- Supplementary measures
- Pulmonary rehabilitation Â
- Long-term oxygen therapy – indications below
- Lung transplant (option for some patients)
Now time to test yourself
What is the difference between hypoxia and hypoxaemia?
Oops! This section is restricted to members.
What is long-term oxygen therapy (LTOT)? What are the indications?
Oops! This section is restricted to members.
What is ambulatory oxygen therapy? What are the indications?
Oops! This section is restricted to members.
Try some OSCE exam stations!
- Pulmonary fibrosis exam
- Interstitial lung disease
- More here