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Prognosis

Authoring team

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.

Reference:

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