Last reviewed 07/2019

Only a small proportion respond to treatment, though in some cases the disease does not progress.

Overall the prognosis is poor:

  • median survival is approximately 3 years
  • the range of survival is 1-20 years
  • there is a 10-fold increase in the incidence of bronchogenic carcinoma

Lung transplantation is the only therapy which has been proven to increase the long-term survival of patients.