Pathology: Fibrosisand remodeling of the interstitium following chronic inflammation
Aetiology: Seebelow
Symptoms: Shortness of breathon exertion, dry cough
Signs: Dyspnoea, hypoxia, clubbing,late inspiratory crackles on auscultation, raised JVP and peripheral oedema
Investigations: CT: Reticulation and honeycombingsuggest fibrosis
Ground glass changes suggestsinflammation
Spirometry, lung volumes and gas transfer: classically restrictive pattern
Lung biopsy: Bronchoscopy or surgical biopsy
Treatment: Avoidance ofaetiology
Steroids: more likely to help if there is moreinflammation and less fibrosis
Immunosuppressants: e.g. azathioprine
Lung transplantation: Considered in the young withadvanced unresponsive disease
Palliation: Important in end stage disease
Complications: End-stagefibrosis
Prognosis: Overall5-year survival rate is around 50%
Figure 2.9 Bilateral lower zonefibrosis
