LUNG TRANSPLANTATION IN PATIENTS WITH SYSTEMIC-DISEASES - AN 11-YEAR EXPERIENCE AT PAPWORTH-HOSPITAL

Background: The presence of a systemic disease has traditionally been considered a contraindication to lung transplantation. Methods: We pre sent a retrospective review of 19 patients undergoing lung transplanta tion for end-stage pulmonary disease associated with a systemic illnes s since 1984. There were 11 male and 8 female patients, aged from 23 t o 59 years (median 43 years) with end-stage pulmonary involvement by s arcoidosis (11 patients), Langerhan's cell histiocytosis (three patien ts), systemic vasculitis (four patients: three with systemic lupus ery thrematosis, one with Churg-Strauss), and common variable immunodefici ency (one patient). Ten patients received a heart-lung transplant, and eight patients received a single lung transplant. One patient underwe nt single lung transplantation after an earlier heart-lung transplant. Results: The 30-day mortality was 5.3%. Nine patients died overall. T wo of these had systemic lupus erythrematosis with anticardiolipin ant ibodies and died from complications of their underlying vasculitis. Th e mean 1- and 2-year actuarial survivals for all patients were 71% (st andard error +/- 10.8%) and 64% (standard error +/- 11.9%), respective ly. All patients surviving longer than 3 months achieved an improvemen t in functional status to New York Heart Association class I or II, an d a significant increase occurred in mean forced expiratory volume in 1 second and forced vital capacity. Disease recurrence without clinica l significance occurred in two patients with sarcoidosis. Of the nine patients who died, seven had autopsies and none showed evidence of dis ease recurrence in the lungs. Conclusions: Patients with systemic dise ases can be considered for lung transplantation and each case should b e judged on its individual merits. However, patients with systemic lup us erythrematosis (particularly when associated with anticardiolipin a ntibodies) should probably not be offered lung transplantation because they are likely to develop further complications of their underlying vasculitis.