PROLONGED SURVIVAL AFTER HEART-LUNG TRANSPLANTATION IN SYSTEMIC LUPUS-ERYTHEMATOSUS

Patients with multisystem involvement of connective tissue disorders a re generally excluded from consideration for heart-lung and lung trans plantation because of profound donor organ shortages. A 23-year-old wo man with systemic lupus erythematosus (SLE) was referred for evaluatio n of severe, progressive pulmonary hypertension. She underwent an unev entful heart-lung transplant and received cyclosporine A, azathioprine , and prednisone on a long-term basis. Bronchiolitis obliterans result ed in the development of moderate airflow obstruction 18 months after transplantation, but the process was stabilized with augmented immunos uppression consisting of high-dose parenterally administered corticost eroids, and subsequently a course of antithymocyte globulin. Four year s after transplant, despite the persistence of reduced complement leve ls, the patient remains functionally well without clinical manifestati ons of SLE. This patient's long-term successful outcome indicates that connective tissue disorders such as SLE do not necessarily represent absolute contraindications to heart-lung and lung transplantation.