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Aetiological classification of pulmonary fibrosis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Congenital causes:

  • neurofibromatosis
  • Gaucher's disease
  • Niemann-Pick disease
  • tuberous sclerosis

Chemical or dust inhalation:

  • organic dusts e.g. moulds, avian proteins
  • mineral dusts e.g. silica, coal dust, asbestos
  • chemicals e.g. chlorine, nitrogen dioxide

Unknown causes:

  • cryptogenic fibrosing alveolitis
  • idiopathic haemosiderosis
  • histiocytosis X
  • sarcoidosis


  • bleomycin, cyclophosphamide, methotrexate, hydralazine, busulphan, amiodarone

Iatrogenic and poisoning:

  • radiation pneumonitis
  • oxygen toxicity
  • paraquat poisoning

Pulmonary involvement in other disease:

  • rheumatoid arthritis
  • ankylosing spondylitis
  • Sjogren's syndrome
  • systemic sclerosis
  • dermatomyositis

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