Key points

  • Among the 300-plus interstitial lung diseases, the most important are sarcoidosis, idiopathic pulmonary fibrosis, extrinsic allergic alveolitis, interstitial lung disease as a feature of connectivetissuedisease,drug-induced interstitial lung disease and pneumoconiosis.
  • Only about one in three cases of interstitial lung disease has a known cause.
  • Survival rates at 5 years range from 20% for idiopathic pulmonary fibrosis to almost 100% for cryptogenic organising pneumonia.
  • The highest mortality rates are about 2.5 per 100 000 people per year, seen in the British Isles, Scandinavia, the Netherlands and Spain.
  • The management of interstitial lung disease of known aetiology is, in the first place, prevention and cessation of exposure. For interstitial lung disease of unkown aetiology, treatment consists mainly of antifibrotic and anti- inflammatory drugs.

See the entire Interstitial lung diseases Chapter