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.