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.
Chronic progressive shortness of breath
Signs of pulmonary fibrosis
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)
Kyphosis (ankylosing spondylitis)
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
NB: fibrosis is permanent and there is no cure.
Corticosteroids may help in acute exacerbations
Antifibrotics (pirfenidone/nintedanib) may be used in idiopathic pulmonary fibrosis
Treat/withdraw cause if possible (may include corticosteroids and immunosuppressants)
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?
What is long-term oxygen therapy (LTOT)? What are the indications?
What is ambulatory oxygen therapy? What are the indications?