Pulmonary fibrosis

Causes include:

  • chemical or dust inflammation
  • idiopathic (idiopathic pulmonary fibrosis or cryptogenic fibrosing alveolitis)
  • local fibrosis from infection or infarction
  • various congenital syndromes – neurofibromatosis, Gaucher’s disease, Niemann-Pick disease, tuberous sclerosis.
  • various acquired inflammatory conditions – RA, AS, Sjogren’s, systemic sclerosis, dermatomyositis
  • various meds – methotrexate, amiodarone, hydralazine, cyclophosphamide, bleomycin, busulphan
  • some other illnesses – sarcoidosis, idiopathic haemosiderosis, histiocytosis X
  • radiation
  • more obscure forms of toxicity – oxygen toxicity, paraquat poisoning


  • Gradually progressing exertional dyspnoea
  • Dry cough
  • Systemic symptoms in some cases – weight loss, low-grade fevers, fatigue, arthralgia/myalgia


  • Stop smoking, if applicable
  • Inhaled steroids: improve symptoms in about half of cases and lung function in about a quarter. No evidence as to whether they prolong life.
  • Oxygen therapy if sats <88% (I got that from an article on US guidelines – haven’t checked whether recommendation the same here, but that should give a general idea)
  • Immunosuppressive treatment – not enough evidence to say whether helpful or not.


Sources: GP Notebook and emedicine article

Learning objective: Seeing patient in upcoming session with newly diagnosed PF, want to refresh my memory on the basics enough to be able to have a helpful conversation.



About Dr Sarah

I'm a GP with a husband and two young children.
This entry was posted in Credits 2016, Respiratory, Uncategorized. Bookmark the permalink.

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