title: Long-term follow-up after nonmyeloablative allogeneic hematopoietic stem cell transplantation for systemic sclerosis.
abstract: Systemic sclerosis (SSc) characteristically consists of fibrotic changes in various organs, and immunological abnormality is the main cause of the disease. Although high-dose immunosuppressive therapies with autologous or allogeneic hematopoietic stem cell support can reverse the disease course, they have a high treatment-related mortality. We report the successful use of nonmyeloablative allogeneic hematopoietic stem cell transplantation (HSCT) for SSc. A 40-year-old woman with diffuse scleroderma and interstitial pneumonia underwent allogeneic peripheral blood stem cell transplantation from an HLA-identical sibling after conditioning with low-dose total-body irradiation and fludarabine. Prophylaxis for graft-versus-host disease (GVHD) consisted of cyclosporine and mycophenolate mofetil. No infection or acute GVHD developed. One year after transplantation, the patient developed membranous glomerulopathy caused by chronic GVHD that was successfully treated with prednisolone. The patient's skin score decreased dramatically, and her pulmonary function is stable 4 years after transplantation. Nonmyeloablative allogeneic HSCT may be more effective than conventional therapies for SSc.
authors.affiliation: Department of Immunobiology and Neuroscience, Medical Institute of Bioregulation, Kyushu University, Beppu, Oita, Japan.
authors.forename: Motoaki
authors.initials: M
authors.lastname: Shiratsuchi
authors.forename: Seiichi
authors.initials: S
authors.lastname: Motomura
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Abe
authors.forename: Satoshi
authors.initials: S
authors.lastname: Shiokawa
authors.forename: Junji
authors.initials: J
authors.lastname: Nishimura
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D012595:Scleroderma, Systemic / Q000628:therapy*