Interstitial lung disease is a heterogeneous group of subacute or chronic illnesses, which may lead to respiratory failure and death in a large number of patients. Idiopathic pulmonary fibrosis is the most common form of such a diseases.
From January 1991 to April 2005, 243 patients affected by pulmonary fibrosis were referred to lung transplantation waiting list: 110 (45%) died, 90 (37%) underwent lung transplantation, 22 (9%) are still enrolled and 21 (9%) were suspended from the list. Actuarial survival of patients on waiting list (medical therapy) is summarized on table 1.
90 patients affected by pulmonary fibrosis underwent 94 transplantations, with 4 patients undergoing retransplantation. There were 80 (89%) single lung transplants with 45 twinning procedures, 9 (10%) double lung transplants and 1 (1%) heart-lung transplant. Cardiopulmonary by-pass was used in 11% of patients (mean time 163±84 min), total ischemic time was 242±86 minutes. Median of mechanical ventilation was 2 days; median ICU and hospital stay was 5 and 17 days, respectively. Postoperative mean FEV1 increased from a preoperative level of 46% of the predicted value to 69% at 1 month and 74% at 12 months. Actuarial survival after lung transplant is summarized on table 2. The incidence of postoperative complications was 1,02 patient. Airway complications occurred in 20 patients (23%). Comparison of survival rates between transplanted patients and patients on waiting list are highly significant (p<0,000001).
Lung transplantation is an excellent but, by now, the only one therapeutic option for patients affected by end-stage pulmonary fibrosis. Currently medical treatment of pulmonary fibrosis with single or combined immunosuppressive therapy has not been found to improve survival of these patients. Lung transplantation is also effective in improving respiratory function and returning many patients to active life.
The high mortality of patients affected by pulmonary fibrosis on waiting list and the lack of donors for lung transplant impose the search for new therapeutic modalities in pulmonary fibrosis.
Carlo Banfi, None.