Interstitial Lung Disease

Interstitial Lung Disease

Pulmonary Fibrosis

Pathology:                    Fibrosisand remodeling of the interstitium following chronic inflammation

Aetiology:                     Seebelow

Text Box: Causes Of Pulmonary Fibrosis

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















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%

Text Box: Upper Zone Fibrosis Mnemonic:
Extrinsic Allergic Alveolitis
Ankylosing Spondylitis













Figure 2.9 Bilateral lower zonefibrosis

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