title: Haematopoietic stem cell transplantation for vasculitis including Behcet's disease and polychondritis: a retrospective analysis of patients recorded in the European Bone Marrow Transplantation and European League Against Rheumatism databases and a review of the literature.
abstract: OBJECTIVE
  To evaluate the feasibility of haematopoietic stem cell transplantation (HSCT) in vasculitis.
  
  
  METHODS
  This is a retrospective analysis of patients who had received HSCT for vasculitic diseases and have been reported to the European League Against Rheumatism autoimmune disease or European Bone Marrow Transplantation ProMISe databases. Information about the disease and outcome was obtained by a questionnaire sent to the referring centres. Response of the disease to HSCT was defined as partial or complete responses according to the ability to reduce immunosuppression after HSCT. In addition, the Medline database was searched for reports on HSCT in patients with vasculitis.
  
  
  RESULTS
  Detailed information was obtained for 15 patients, whose median age at HSCT was 37 years. The diagnoses were cryoglobulinaemia in four patients, Behçet's disease in three patients, Wegener's granulomatosis in three patients, and undifferentiated vasculitis, Churg-Strauss angiitis, polychondritis, Takayasu arteritis and polyarteritis nodosa in one patient each. 14 patients received autologous HSCT and 1 an allogeneic HSCT as the first transplant. In three patients, further transplantation was given because of relapse. The overall response, including all consecutive transplantations (HSCT/patient, n = 1-3, median 1.3) to HSCT, was 93%, with 46% complete responses and 46% partial responses; median (range) duration of response at the time of reporting was 45 (16-84) months. Three patients died, one from advanced disease, one from cancer and one from graft-versus-host disease. The Medline search showed five other patients who were effectively treated with HSCT for vasculitic diseases.
  
  
  CONCLUSIONS
  This retrospective study suggests that autologous HSCT is feasible for vasculitis. Its value remains to be tested in prospective controlled studies.
authors.affiliation: Department of Rheumatology, University of Basle, Petersgraben 4, CH-4031 Basle, Switzerland. tdaikeler@uhbs.ch
authors.forename: Thomas
authors.initials: T
authors.lastname: Daikeler
authors.forename: Ina
authors.initials: I
authors.lastname: Kötter
authors.forename: Chiara
authors.initials: C
authors.lastname: Bocelli Tyndall
authors.forename: Jane
authors.initials: J
authors.lastname: Apperley
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Philippe
authors.initials: P
authors.lastname: Guardiola
authors.forename: Alois
authors.initials: A
authors.lastname: Gratwohl
authors.forename: Esa
authors.initials: E
authors.lastname: Jantunen
authors.forename: Alberto
authors.initials: A
authors.lastname: Marmont
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Porretto
authors.forename: Maurizio
authors.initials: M
authors.lastname: Musso
authors.forename: Britta
authors.initials: B
authors.lastname: Maurer
authors.forename: Nadia
authors.initials: N
authors.lastname: Rinaldi
authors.forename: Riccardo
authors.initials: R
authors.lastname: Saccardi
authors.forename: Alan
authors.initials: A
authors.lastname: Tyndall
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000601:surgery; D002357:Cartilage Diseases / Q000601:surgery; D016208:Databases, Factual; D005060:Europe; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000706:statistics & numerical data*; D006801:Humans; D007165:Immunosuppression Therapy; D016239:MEDLINE; D008297:Male; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies; D014182:Transplantation, Autologous; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D014657:Vasculitis / Q000601:surgery*