Prolonged survival after heart-lung transplantation in systemic lupus erythematosus

Patients with multisystem involvement of connective tissue disorders are generally excluded from consideration for heart-lung and lung transplantation because of profound donor organ shortages. A 23-year-old woman with systemic lupus erythematosus (SLE) was referred for evaluation of severe, progressive pulmonary hypertension. She underwent an uneventful heart-lung transplant and received cyclosporine A, azathioprine, and prednisone on a long-term basis. Bronchiolitis obliterans resulted in the development of moderate airflow obstruction 18 months after transplantation, but the process was stabilized with augmented immunosuppression consisting of high-dose parenterally administered corticosteroids, and subsequently a course of antithymocyte globulin. Four years after transplant, despite the persistence of reduced complement levels, the patient remains functionally well without clinical manifestations 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.