title: Autologous stem cell transplantation improves microcirculation in systemic sclerosis.
abstract: BACKGROUND
  In systemic sclerosis (SSc) reduced capillary density decreases blood flow and leads to tissue ischaemia and fingertip ulcers. Nail fold videocapillaroscopy (NVC) is a diagnostic and follow-up parameter useful to evaluate the severity, activity and the stage of SSc microvascular damage. Autologous haemopoietic stem cell transplantation (HSCT) is a new treatment for patients with severe diffuse cutaneous systemic sclerosis (dcSSc) refractory to conventional therapies. We aimed to evaluate the improvement of microvasculature after HSCT using NVC.
  
  
  METHODS
  A total of 16 patients with severe dcSSc with a "late" videocapillaroscopy pattern underwent an immunesuppressive treatment: 6 were treated with HSCT and 10 with monthly pulse cyclophosphamide (CYC) 1 g for 6 months and then orally with 50 mg/day for further 6 months. NVC was performed before and after 3 months from the beginning of each treatment and then repeated every 3 months.
  
  
  RESULTS
  In all patients, before HSCT NVC showed large avascular areas and ramified capillaries and vascular architectural disorganisation ("late" pattern). At 3 months after HSCT, the NVC pattern changed from "late" into "active", showing frequent giant capillaries (>6/mm) and haemorrhages, absence of avascular areas and angiogenesis phenomena; 1 year after HSCT, microvascular abnormalities were still in the "active" pattern. In patients treated with CYC, no NVC modifications were observed during 24 months of follow-up and the pattern always remained "late".
  
  
  CONCLUSIONS
  These results indicate that HSCT with a high dose CYC regimen may foster vascular remodelling, while CYC at lower doses and with a chronic regimen does not influence the microvasculature.
authors.affiliation: Department of Biomedicine, Section of Rheumatology, DENOthe Center, AOUC Florence, Viale Pieraccini 18, 50139 Florence, Italy. irene.miniati@unifi.it
authors.forename: I
authors.initials: I
authors.lastname: Miniati
authors.forename: S
authors.initials: S
authors.lastname: Guiducci
authors.forename: M L
authors.initials: ML
authors.lastname: Conforti
authors.forename: V
authors.initials: V
authors.lastname: Rogai
authors.forename: G
authors.initials: G
authors.lastname: Fiori
authors.forename: M
authors.initials: M
authors.lastname: Cinelli
authors.forename: R
authors.initials: R
authors.lastname: Saccardi
authors.forename: S
authors.initials: S
authors.lastname: Guidi
authors.forename: A
authors.initials: A
authors.lastname: Bosi
authors.forename: A
authors.initials: A
authors.lastname: Tyndall
authors.forename: M
authors.initials: M
authors.lastname: Matucci-Cerinic
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D004334:Drug Administration Schedule; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008833:Microcirculation; D020678:Microscopic Angioscopy / Q000379:methods; D008875:Middle Aged; D009262:Nails / Q000098:blood supply; D011446:Prospective Studies; D012039:Regional Blood Flow; D045743:Scleroderma, Diffuse / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery; D018709:Statistics, Nonparametric; D014182:Transplantation, Autologous; D014741:Video Recording
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide