Reading Time:
Pathology
Fibrosis and remodeling of the interstitium following chronic inflammation
Aetiology
Upper Zone Fibrosis Lower Zone Fibrosis
Berylliosis Connective tissue disorders:
Radiation SLE, RA, Scleroderma
Extrinsic Allergic Alveolitis
Ankylosing Spondylitis Drugs:
Sarcoidosis Amiodarone, nitrofurantoin, methotrexate,
Tuberculosis bleomycin, cyclophosphamide
Occupational lung disease:
Asbestosis. silicosis
Signs
Dyspnoea, hypoxia, clubbing, late inspiratory crackles on auscultation, raised JVP and peripheral oedema
Symptoms
Shortness of breath on exertion, dry cough
Investigations
CT: Reticulation and honeycombing suggest fibrosis
Ground glass changes suggests inflammation
Spirometry, lung volumes and gas transfer: classically restrictive pattern
Lung biopsy: Bronchoscopy or surgical biopsy
Treatment
Avoidance of aetiology
Steroids: more likely to help if there is more inflammation and less fibrosis
Immunosuppressants: e.g. azathioprine
Lung transplantation: Considered in the young with advanced unresponsive disease
Palliation: Important in end stage disease
Prognosis
Overall 5-year survival rate is around 50%
End-stage fibrosis
Key Facts
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key Images
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key References
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.