- 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
- 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.