title: Prolonged survival after heart-lung transplantation in systemic lupus erythematosus.
abstract: 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.
authors.affiliation: Respiratory Division, Royal Victoria Hospital, McGill University, Montreal, Quebec, Canada.
authors.forename: R D
authors.initials: RD
authors.lastname: Levy
authors.forename: A J
authors.initials: AJ
authors.lastname: Guerraty
authors.forename: M H
authors.initials: MH
authors.lastname: Yacoub
authors.forename: R
authors.initials: R
authors.lastname: Loertscher
mesh_terms: D000328:Adult; D005260:Female; D006085:Graft Survival; D016041:Heart-Lung Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000601:surgery; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D011652:Pulmonary Circulation
chemical_list: D007166:Immunosuppressive Agents